International Journal of Neurooncology

: 2021  |  Volume : 4  |  Issue : 2  |  Page : 84--110

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Study of cortactin-CD44-mediated meningioma progression

Shubhangi B, P. Prakash Babu, Manas Panighahi; University of Hyderabad, Hyderabad, Telangana, India

Objective: Cluster of differentiation 44 (CD44) is a homing cell adhesion molecule involved in cell-cell and cell-extracellular matrix communications. It is largely involved in proliferation, mortality, and intracellular signaling for cell growth. Cortactin (CTTN) is a cortical actin-binding protein that plays role in lamellipodia formation, which has an important function in regulating directed cell motility. Here we studied downstream effectors of CD44 and the mechanism by which CTTN ultimately modulates the invasion and metastasis in meningioma. CTTN causes actin cytoskeleton remodeling to promote epithelial to mesenchymal transition. It binds different proteins and participates in different mechanisms that induce the progression of epithelial to mesenchymal transition which is important for cancer metastasis. This study aimed to identify CTTN-CD44-mediated epithelial-mesenchymal transition in the progression of meningioma.

Methods Used: Patient samples (n = 32), PCR, RT-PCR, Western blot (WB), immunohistochemistry (IHC), immunofluorescence (IF), and immunoprecipitation (IP).

Results: PCR and RT-PCR results show that CTTN and CD44 gene expression increased from control arachnoid to grade III of meningioma. Following it, WB results indicate its protein expression also increases in higher grades as compared to lower grades and control samples. Immunohistochemical staining shows a cytoplasmic expression of CTTN and nucleocytoplasmic expression of CD44 in higher grades of meningioma. IF staining shows they are colocalized with each other and increased expression during the progression of meningioma. IP results resemble IF results and indicate both of these proteins are interacting with each other and help in the progression of meningioma.

Conclusion: Our study revealed that assessment of CTTN interaction with CD44 in meningioma suggests their role in meningioma progression and the significant correlation between CD44 and CTTN could provide insights into their critical role in epithelial to mesenchymal transition in meningioma.

A survival analysis of high-grade gliomas in sub-Himalayan population including the times of lockdown during COVID-19 pandemic: A single-institutional experience

Pooja Kalra, Meenu Gupta, Vipul Nautiyal, Ranjeet Kumar, Sanjeev Pandey, Nazia Shirazi, Brijesh Tiwari, Mushtaq Ahmad; Swami Rama Himalayan Institute

Background and Objectives: High-grade gliomas are most common, accounting for more than 70% of primary brain tumors, they are categorized as Grade III and IV and they have a high mortality rate with a poor prognosis. How we should adopt the clinical practice in neuro-oncology during the COVID-19 pandemic is another area of scientific exploration. Neuro-oncology population is having cognitive decline and utilization of telemedicine oncology communication has limitations in this group of population. Hypofractionated radiotherapy protocols can be easily utilized in high-grade gliomas during the COVID-19 pandemic.

Materials and Methods: Retrospective and prospective analysis of 147 patients with diagnosis of high-grade gliomas who were treated at our department between January 2009 till December 2020 including COVID-19 pandemic lockdown time was done. Age, gender, KPS, symptoms, site of lesion and laterality, extent of surgery, and use of concurrent temozolomide were evaluated using univariate and multivariate analysis. Overall Survival was determined using the Kaplan–Meir method.

Results: Male-to-female ratio was 2:1. Glioblastoma multiforme is the most common brain tumor (82.3%) in all high-grade gliomas. The left side of brain with frontal lobe is the most common site of brain tumor. Near-total or total excision was done in 36.7% of cases. The median dose of EBRT delivered was 60 Gy. 43 (29.2%) patients of total patients were treated with concurrent and adjuvant temozolomide during COVID-19 pandemic lockdown time due to severe neurological symptoms. All patients were tested for COVID-19 before surgery. The median overall survival was 15.9 months. The 6 months overall survival was 93.8%, 1 year OS was 67.8%, 2-year OS was 19.2%%, and 3-year OS was 6.4%. Only 1 patient survived for more than 5 years. Out of 43 patients treated during COVID pandemic time, 62.7% are alive and on follow-up. Quality of life data will be presented at the conference

Conclusion: The results of our survival analysis of high-grade gliomas demonstrated the benefit of adding radiation with concurrent and adjuvant temozolomide in high-grade gliomas including COVID-19 during lockdown time. Hypofractionated radiotherapy with concurrent temozolomide is safe during the COVID-19 pandemic.

Analysis of survival outcomes in pediatric sub-Himalayan population with primary CNS malignancy: A single-center experience

Shalini Singh, Meenu Gupta, Vipul Nautiyal, Ranjeet Kumar, B.P. Kalra, Kunal Das, Nadia Shirazi, Mushtaq Ahmad; Swami Rama Himalayan University

Background and Objectives: Pediatric CNS tumor encompasses a wide range of diagnosis from benign to malignant. Pediatric population requires a multidisciplinary approach including a play therapist, supportive anesthetist, and neuro-oncologist. Early diagnosis and appropriate interventions can result in improvement in quality of life.

Materials and Methods: A single-institution retrospective data collection of brain tumors in pediatric patients from January 2009 to February 2021 who received radiotherapy at our center.

Inclusion Criteria: Over 18 years of age, primary neurological tumors, low- to high-grade histology, patients who received radiotherapy in any setting at our center and completed treatment.

Exclusion Criteria: Over 18 years of age, metastatic neurological disease, and any brain mass without histology.

Results: Total patients (n) = 36; lowest age being 3 years, with a male-to-female ratio 1.5:1; histology-meduloblastoma 27.7%, astrocytoma 16.6%, craniopharyngioma 16.6%, GBM 13.8%, oligodendroglioma 8.3%, glioma 8.3%, ependymoma 2.7%, embryonal tumor 2.7%, and meningioma 2.7%. Common site encountered: suprasellar region 19.4%, cerebellar 16.6%, posterior fossa 16.6% and frontal lobe 16.6%. 91.6% of patients underwent surgery with radical intent and 5.5% underwent stereotactic biopsy. RT schedules: 30–60 Gy in multiple fractionation. 27.7% patients were treated during COVID-19 pandemic. 61.6% patient are alive and currently on follow-up. Radiation demonstrated survival benefit over surgery alone.

Conclusion: Brain tumor management is a challenge in pediatric patients due to un-cooperability, inability to correctly follow verbal and visual commands; however, combined treatment modality results in improved outcomes.

Efficacy of whole-brain RT among the subtypes of metastatic breast cancer

Shija Merrin Mathew, Uday Krishna, V. Lokesh; Kidwai Memorial Institute of Oncology

Objectives: To understand the effect of whole brain radiation (WBRT) in terms of age, neurological performance, radiological improvement, and the overall survival

Materials and Methods: Thirty-four patients with metastatic breast carcinoma who presented with brain metastasis to the Department of Radiation Oncology at the Kidwai Memorial Institute of Oncology and subjected to Whole Brain Radiotherapy/ Focal RT were taken into the study. Patients were characterized on the Molecular subtype, Age, Timeline of Presentation and Radiological Findings. The efficacy of WBRT was assessed based on the Neurological, Radiological and Overall Survival among the four subtypes of MBC

Results: Among patients under Luminal A, 58% underwent surgery followed by WBRT and OS fared better as compared to patients with WBRT alone with no distant recurrences. However, in the patients with luminal B/HER2 amplified subgroup, 18% received Herceptin and Lapatinib and 33% received Herceptin alone while 22% received Lapatinib alone. OS was superior over WBRT alone in these patients and WBRT lead to exaggerated radiation necrosis in those who took biological therapy and RT concurrently with reduced KPS. In patients with basal subtype had poor KPS, whereas OS remained poor despite WBRT.

Conclusions: Lesions being amenable for surgery, focal RT to postop cavity alone may be considered in patients with Luminal A. WBRT plus boost for those unfit for surgery. Addition of TKI ± oral chemo for all patients with Luminal B and HER 2 amplified to Focal RT (single/oligo mets) or WBRT (multiple mets). Triple-negative metastatic breast cancer patients present with poor KPS thus can be considered for choice of BSC/WBRT on a case basis.

Thyroid and hippocampal doses in conformal cranial radiotherapy and its association with tumor location: A phantom dosimetric study

Poorva Vias, Shikha Goyal, Ranjit Singh, Renu Madan, Vrinda Singla; PGIMER, Chandigarh, India

Purpose or Objective: Patients who receive radiotherapy for benign brain tumors or low-grade gliomas live long enough to develop quality of life (QOL) issues due to memory and are prone to develop radiation-induced second cancers. Traditionally, critical structures evaluated include neural optic structures but data on thyroid and hippocampal doses are scarce. We attempted to evaluate doses to these structures in relation to tumor location within the brain.

Material and Methods: Axial CT images of CIRS Atom phantom (900 Asbury Ave, Norfolk, Virginia) were acquired from the CT simulator (Philips Bigbore 4DCT) at 1 mm slice thickness from vertex till carina, and co-registered with a normal volumetric contrast-enhanced magnetic resonance study to help delineate lobar anatomy of brain, hippocampus, and thyroid. 3D CRT target volumes in each of the four cerebral lobes, brainstem, and cerebellum were delineated and treatment planning was performed on computer-based treatment planning system (TPS), Eclipse (V 11.0, Varian Medical System, Palo Alto, California) used analytical anisotropic algorithms (V11.0.31). 3D CRT plans (54 gray in 27 fractions over 5.5 weeks) were generated for all six volumes for Trilogy‐5823 (Varian Medical System, Palo Alto, California) with and without multileaf collimators (MLC). The primary objective was to study the planned dose to the delineated organs at risk with respect to tumor location and use of MLC for beam shaping.

Results: Use of MLC did not impact PTV coverage but reduced maximum and mean doses to critical structures close to PTV. Thyroid doses were highest for posterior fossa and brainstem lesions, and lowest for parietal lesions; MLC use led to a significant reduction of thyroid doses in posterior fossa, brainstem and temporal lobe lesions. Hippocampal doses were lowest in occipital and parietal lesions and highest for posterior fossa lesions; use of MLC reduced hippocampal doses for all sites except occipital tumors. Table 1 lists the target volumes and various target and OAR dosimetric parameters. Results of TLD dosimetry would be reported later.

Conclusion: Hippocampal sparing may be relevant in posterior fossa or temporal lobe lesions. In absence of more sophisticated techniques, even MLC use in 3DCRT may lead to ~10% reduction in hippocampal dose. 3DCRT plans with MLC shielding also reduce scattered dose to thyroid, especially in posterior fossa or brainstem irradiation, and may be relevant in reducing second malignancy risk in low-grade tumors. TLD verification can help dose confirmation, especially for superficial structures.

Quantification of CfDNA levels and IDH-1 mutation in adult diffuse glioma for diagnosis and prediction of therapy

Adil Husain, Nuzhat Husain, Saumya Shukla, Rahat Hadi, Rohini Khurana, Madhup Rastogi, Avnish Sahu, Mohammed Haris Siddiqui; Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background/Objectives: Circulating-free DNA (cfDNA) quantification and profiling have emerged as novel noninvasive diagnostic and prognostic biomarkers in solid tumors. Change in CfDNA level may provide information for tumor progression and treatment response. This study was designed to quantify the cfDNA and correlated it with the treatment response. IDH1 Mutation in cfDNA and respective FFPE tissue DNA was also evaluated.

Materials and Methods: The study group comprised 27 biopsy-proven cases of adult gliomas including 6 grade II, 5 grade III, 16 grade IV gliomas, and 15 healthy individuals. CfDNA was extracted from serum by using the ChargeSwitch gDNA 1-mL Serum Kit (Invitrogen, USA #CS11040) according to the manufacturer’s instructions in 27 serial ADG cases and 15 healthy individuals. CfDNA was quantified by quantitative SYBR Green real-time PCR using β-globin gene and compared with standard curve plotted by TaqMan Control Human Genomic DNA (Applied Biosystems, USA). IDH1 mutation (R132H) was determined by CAST qPCR assay and Next-generation sequencing (NGS).

Results: The cfDNA (mean ± SD) level was higher in pre-treatment samples of adult diffuse glioma (349.19 ± 618.14) as compared to the normal controls (90.79 ± 52.73). CfDNA level was significantly higher in non-responders as compared to responders (P =0.015). The cfDNA level was significantly associated in patients with treatment response in IDH1 mutated, P53 mutated, and ATRX no loss (P = 0.04, 0.01, and 0.02), respectively. CAST PCR assay detected IDH1 mutation in 12/24 (50%) FFPE tissue DNA while in cfDNA could not be detected (0/24). IDH1 mutation in follow-up cfDNA samples could not be detected by NGS also.

Conclusions: Adult diffuse gliomas have a significantly high level of cfDNA in sera. CfDNA level was significantly associated with treatment response. A significant decrease in the titers was evident in the cases of ADG with IDH mutation status. CAST PCR assay could not detect the IDH1 (R132H) mutation in cfDNA.

Whorling�sclerosing meningioma: Twin case reports of a rare tumor

Pranav Shirbhate, Ashvini Kolhe, Asha Shenoy; Seth GSMC and KEM Hospital

Introduction: Whorling–sclerosing meningioma (WSM) is a very rare distinct histologic subtype of meningioma with a benign course. Davidson et al. described sclerosing in 1989, while Haberler et al. in 2002 presented WSM as a distinct variety of meningioma. In WSM, whorling–sclerosing structures are formed by typical paucicellular or acellular collagen in whorls, which form the majority of tumor volume.

Case Report: Case 1: A 25-year-old man presented with 6 weeks history of tingling numbness over his left face, diplopia, and hypoesthesia in V1 distribution.

MRI of the brain showed a 20 mm x 21 mm x 20 mm heterogeneous extra-axial lesion in left prepontine cistern with a mass effect on 5th cranial nerve.

Tumor was surgically excised and tissue was received for intraoperative diagnosis. However, the tissue did not spread. Final diagnosis was rendered subsequently on routine processing.

Case 2: A 41-year-old man came with left frontoorbital SOL since 1 month with headache.

MRI of the brain showed T2 hypointense lesion in left orbit extending up to left cavernous region. The patient had undergone surgery for similar lesion 25 years back.

After surgical resection of the tumor, we received multiple firm gray-white tissue bits aggregating to 4.5 cm x 3.0 cm x 0.3 cm. Tissue was processed routinely.

Microscopy: Histopathological examination of both the above cases revealed tumor full of bands of collagenous tissue arranged in concentric mostly sclerosing whorls. Few psammoma bodies were seen. Periphery showed meningothelial cells in clusters and small lobules. No mitosis/necrosis or brain invasion seen. Other than above case 1 also showed few cells with clear cytoplasm, vesicular nuclei, and showing prominent nucleoli. Diagnosis of whorling-sclerosing meningioma (WSM) was given.

Postoperatively, the patients are doing well to date with no evidence of immediate complication.

Conclusion: A neuropathologist must be aware of the morphology of this rare variant of meningioma to render correct diagnosis which would, in turn, affect therapeutic decision.

Contralateral approach in surgical management of spinal meningioma in tertiary care center

Kodali Sandhya, Manas Panigrahi, YBVK Chandrasekhar, Kodali Sandhya, Manas Panigrahi, YBVK Chandrasekhar; Krishna Institute of Medical Sciences, Hyderabad, Telangana, India

Introduction: Spinal meningiomas are about 12%–15% of all meningiomas. Recurrence in anterior and lateral located meningiomas is due to incomplete excision of dura. Contralateral approach aids in the complete excision of dura.

Objectives: The aim of this study was to evaluate the contralateral approach in surgical procedure and postoperative outcome.

Materials and Methods: Retrospective analysis was done from 2012 to 2020 in a single tertiary care center. A total of 42 patients have been analyzed for clinical evaluation, surgical procedure, postoperative outcome, complications, recurrence and follow-up.

Results: Predominantly female presentation noted in third and fourth decades. Symptom relief noted in all pts. Four patients operated elsewhere had recurrence and underwent revision surgery in our center. Thoracic region was the most common. Histologically grade 1 and psammatous type were most common. Two patients deteriorated, improved with physiotherapy. In a follow-up range of 3 months to 9 years no recurrence was noted. No mortality noted.

Conclusion: Contralateral approach in spinal meningioma helps in minimal bony dissection, ease of access, and visualization to lesion. It decreases the rate of recurrence.

Downregulation of RAP2B promotes astrocytoma progression by involvement of MiRNA

Neera Yadav, Manas Panigrahi, P. Prakash Babu; University of Hyderabad, Hyderabad, Telangana, India

Glioblastoma (GBM) is highly lethal type of brain cancer, mostly affect the central nervous system. It is found to be most aggressive type of cancer that has ability to migrate away from tumor core to normal cells region cause tumor malignancy (Schwartzbaum, Fisher et al. 2006). Because of improper diagnosis, and inadequate treatment options, GBM has very high mortality and morbidity across the world. So, there is need to find therapeutic interventions of GBM by identifying the molecular and drug candidate. We have selected Ras-related RAP2B protein well reported in various cancers as oncogenic protein. Several study showed that RAP2B promoted proliferation and migration via the activation of ERK signaling in breast cancer. RAP2B is a member of the Ras oncogene family and a novel target of p53 that regulates the p53-mediated pro-survival functions of cells. RAP2B acts as molecular switches and are able to regulate several cellular processes, including adhesion, proliferation, differentiation and apoptosis by modulating a variety of signaling pathways in addition to cell spreading through its interaction with MAP4K4, TNIK, PARG1 and RPIP9. PLC-€ which is activated by interacts with RAP2B facilitates cell growth through activating the Ras-Raf-MAPK/ERK signaling pathway. But the clinical importance of RAP2B regulated by possible involvement of oncogenic mi-RNA in GBM patient. Further, we need to investigate the functional importance of RAP2B in GBM cell line.

Materials and Methods: RAP2B expression was evaluated in human astrocytoma tissue (n = 64) by RT-qPCR, western blotting, immune-histochemistry. RAP2B expression was analyzed with various clinicopathological parameters and TCGA dataset and correlated with GBM patient survibilty during treatment and after therapy.

Results: Our findings revealed down-regulation of RAP2B in malignant astrocytoma associated with pathological grading and patient’s poor survivability corroborating with TCGA dataset. We observed the positive correlation between RAP2B with P53 in GBM tissues which may extort GBM malignancy and poor prognosis.

Conclusions: Altogether, we found down-regulation of RAP2B in malignant GBM patient. Suggested that find the correlation of oncogenic Mi-RNA and RAP2B relation association with malignant GBM patient survibility.

Giant CNS germ cell tumor with spontaneous reduction in size: A case report

SRH Peeran, Baylis Vivek Joseph, Tony Varghese Panicker; CMC, Vellore, Tamil Nadu, India

Introduction: Congenital central nervous system germ cell tumors (GCT) are rare entities and constitute only about 2%–4% of all the CNS tumors with a slight male preponderance. The most common location is in the midline, that is, the pineal region and the suprasellar region. The elevation of tumor markers specific to these tumors is important in the preoperative assessment along with radiological evidence and location. Spontaneous reduction in the size of such a tumor, with raised tumor marker (alpha feto-protein [AFP]), has not been reported before.

Observation: A female neonate was delivered by caesarean section as she was detected to have a left lateral ventricle mass antenatally, on ultrasound neurosonogram, and subsequent fetal MRI, at 37 weeks of gestational age.

After birth, her serum AFP was 2430.0 IU/mL and HCG-BETA was 0.2mIU/ml, which suggested a GCT. Neurosonogram done after birth was suspicious of mild increase in the size of the tumor compared to the fetal MRI (5.6 cm x 3.9 cm and 5.2 cm x 4 cm x 4.5 cm, respectively). She was planned for surgery, but the child was lost to follow-up and came to our OPD 4 months later. CT brain done showed regression of the tumor: 2.5 cm x 2 cm in size. Her AFP levels repeated at four months dropped significantly lower to 43.9 IU/ml and a normal HCG-BETA.

Discussion: High levels of Sr. AFP in a neonate are suggestive of germ cell tumor (yolk sac/endodermal sinus tumor, or teratoma). They normally do not spontaneously regress. In our case, this infant on whom we initially were contemplating surgery was lost to follow-up, and subsequently was found to have regression in the size of the tumor. To the best of our knowledge, there has been no such case reported. She is on follow-up currently.

Conclusion: Spontaneous reduction in the size of an intraventricular tumor with raised Sr AFP in a neonate is an unusual case, not reported in the literature.

Pseudo tumor cerebri in carcinoma cervix “The diagnostic conundrum and a treatment dilemma”

Kiran Kumar BR; Bangalore Medical College and Research Institute

Objectives: Pseudotumor cerebri, commonly known as idiopathic intracranial hypertension and empty sella syndrome, is a rare syndrome that manifests mainly as a consequence of raised intracranial pressure. The diagnosis of this condition is established by exclusion, with the aid of radiological imaging, and examination of cerebrospinal fluid. Due to the aggressive nature of this disease, both signs and symptoms may be intermittent, making definitive diagnosis difficult.

Case Report: The patient was a 60-year-old female with stage III Carcinoma Cervix that was treated with concurrent chemoradiation with weekly cisplatin. She completed the concurrent chemoradiation and developed intermittent seizures, headache and weakness of lower limbs. A diagnosis of pseudotumor cerebri was entertained using the modified Dandy Criteria.

Conclusion: Pseudotumor cerebri should be considered in cancer patients presenting with raised intracranial pressure of sudden onset in the absence of clinical and radiological evidence of brain metastases. We advise early intervention in patients with pseudotumor cerebri to prevent the morbidity.

Clinicopathological Spectrum of leptomeningeal metastases: A 3-year retrospective study

Saloni Pahwa, Anila Sharma, Sunil Pasricha, Meenakshi Kamboj; Rajiv Gandhi Cancer Institute

Introduction: Cytological examination of cerebrospinal fluid is a widely used cost-effective, simple procedure and a reliable routine diagnostic test. CSF cytology helps in detection of inflammatory diseases of the CNS, diagnosis of subarachnoid hemorrhage and the identification of malignant cells in metastatic or rarely primary CNS malignancies. Leptomeningeal metastasis (LM) is estimated to occur in 5% of all patients with cancer. It has a higher propensity to occur in solid tumors compared to hematological malignancies. In view of poor prognosis, early diagnosis may aid in appropriate tumor staging and aggressive therapeutic intervention.

Materials and Methods: All the samples of CSF received for cytological examination and reported as ‘positive for malignant cells’ during the year January 2018 to December 2020 were included in the study. All the cases were routinely evaluated on Neubauer’s chamber, direct smear and a cytospin preparation stained using MGG stain. The clinical records of all samples were retrospectively analyzed.

Results: 87 cases with LM were identified over a 3-year duration. Mean age of presentation was 43 years. Most of the cases (97%) presented with neurological symptoms during the course of treatment while 3 cases (3%) showed LM at the time of first presentation. Metastatic solid malignancies (56%) had a higher incidence of leptomeningeal metastasis compared to hematolymphoid malignancies (40%). Most common solid tumor with involvement of CSF was adenocarcinoma lung (51%) followed by breast carcinoma (37%). Of all the cases of adenocarcinoma lung with LM, EGFR mutant NSCLC were 40% while 8% showed ALK gene rearrangement. 45% of cases of LM in breast cancer were from triple-negative breast carcinomas. Among the hematological malignancies, acute leukemia’s constituted 67% cases of CSF involvement while 34% were systemic NHL’s. 46% of acute leukemia cases that developed CSF involvement were found to have confirmed cytogenetic abnormalities.

Conclusions: With appropriate clinicoradiological correlation, CSF cytology remains the gold standard for identification of malignant cells in cases with already known primary tumor (leptomeningeal dissemination of the disease). In this study, clinical features of both solid and hematolymphoid malignancies were evaluated. The group of solid malignancies included adenocarcinomas lung, breast, gastrointestinal tract, ovarian carcinoma, and renal cell carcinoma.

Clinicopathological spectrum of leptomeningeal metastases: A 3-year retrospective study

Saloni Pahwa, Anila Sharma, Sunil Pasricha, Meenakshi Kamboj; Rajiv Gandhi Cancer Institute

Introduction: Cytological examination of cerebrospinal fluid is a widely used cost-effective, simple procedure and a reliable routine diagnostic test. CSF cytology helps in detection of inflammatory diseases of the CNS, diagnosis of subarachnoid hemorrhage and the identification of malignant cells in metastatic or rarely primary CNS malignancies. Leptomeningeal metastasis (LM) is estimated to occur in 5% of all patients with cancer. It has a higher propensity to occur in solid tumors compared to hematological malignancies. In view of poor prognosis, early diagnosis may aid in appropriate tumor staging and aggressive therapeutic intervention.

Materials and Methods: All the samples of CSF received for cytological examination and reported as ‘positive for malignant cells’ during the year January 2018 to December 2020 were included in the study. All the cases were routinely evaluated on Neubauer’s chamber, direct smear, and a cytospin preparation stained using MGG stain. The clinical records of all samples were retrospectively analyzed.

Results: 87 cases with LM were identified over a 3-year duration. Mean age of presentation was 43 years. Most of the cases (97%) presented with neurological symptoms during the course of treatment while 3 cases (3%) showed LM at the time of first presentation. Metastatic solid malignancies (56%) had a higher incidence of leptomeningeal metastasis compared to hematolymphoid malignancies (40%). Most common solid tumor with involvement of CSF was adenocarcinoma lung (51%) followed by breast carcinoma(37%). Of all the cases of adenocarcinoma lung with LM, EGFR mutant NSCLC were 40% while 8% showed ALK gene rearrangement. 45% cases of LM in breast cancer were from triple-negative breast carcinomas. Among the hematological malignancies, acute leukemia’s constituted 67% cases of CSF involvement while 34% were systemic NHL’s. 46% of acute leukemia cases that developed CSF involvement were found to have confirmed cytogenetic abnormalities.

Conclusion: With appropriate clinicoradiological correlation, CSF cytology remains the gold standard for identification of malignant cells in cases with already known primary tumor (leptomeningeal dissemination of the disease). In this study, clinical features of both solid and hematolymphoid malignancies were evaluated. The group of solid malignancies included adenocarcinoma lung, breast, gastrointestinal tract, ovarian carcinoma and renal cell carcinoma.

Spinal tumors our experience

Sohet Gogia, Vikas Singh, Prakash Shetty, Aliasgar Moiyadi; Tata Memorial Hospital

Objectives: To analyze the demographic data, clinical patterns, association and perioperative outcomes of spinal cord tumors in children and young adults operated using a multimodal approach.

Materials and Methods: This was a retrospective analysis of our surgical database from which 118 patients who were diagnosed with spinal tumor (ST) were analyzed. We looked into the age, symptom duration, and location of tumor, type of tumor, extent of surgery, and adjuncts used intraop, extent of resection, histology type and postop status of these patients.

Results: Out of the 118 patients, 64.4% were young adults and pediatric and rest were adults.

The most common site of tumor in all age groups was cervico thoracic (41.5%).

The most common tumor in pediatric age group were embryonal tumors and astrocytic tumors both (31.2%). In young adults most common tumor pathology was that of ependymal tumors and tumors of spinal nerves both (25%), in contrast to adult where most common tumor pathology was nerve sheath tumors (54.7%).

In pediatric and young adults of those having astrocytic tumor, 50% were high-grade gliomas and 50% were pilocytic tumors. In ependymal cell lineage, 64.2% were low-grade ependymomas. 100% of hemangioblastomas occurred in young adults.

Overall GTR (>95%) was achieved in 49.2%. Of the patients who underwent GTR, majority of them were adults (56.8%). Majority (20.3%) of GTR was seen in tumors arising from spinal nerves (schwannomas) with most common location being thoracic (21.1%).

IOUS was used in 41.5% of cases and out of these cases a GTR resection with help of IOUS was achieved in majority of cases (55.1%).

There were 2 (1.6%) deaths in the postoperative period. Postoperatively 31.3% patients stayed the same, 58.4% improved, and 9.3% deteriorated.

Of the patients in whom a GTR was performed, most of them improved (63.7%).

Conclusions: The tumor pathology is markedly different in the young adult and pediatric population, though the cervical location in all age groups remains most common. In pediatrics astrocytomas and embryonal tumors were most common, in young adults- ependymomas and schwannomas and in adults it is schwannomas. GTR wherever possible with adjuncts like IOUS brings out the best clinical outcome

Splenic volume as a predictor for trends in peripheral blood count and outcomes of radiotherapy in children and young adults with medulloblastoma

Shija Merrin Mathew, Uday Krishna, Naveen T, V. Lokesh; Kidwai Memorial Institute of Oncology

Objectives: To correlate spleen volumes with peripheral blood picture, Overall treatment time (OTT) and outcomes in children and young adults with medulloblastoma to CSI.

Materials and Methods: 27 patients (median age: 9 years [5–23]; M:F = 2:1) with medulloblastoma with median spleen volume: 70.225cc ([43.5 cc-255.27cc]) were divided into low (40–60 cc), moderate (60–80 cc), and high (>80 cc) and further grouped as < 8 years, 8-12 years and >12 years along with risk stratification and subtyping. The median trend of leucopenia during RT. Overall treatment time and response were estimated.

Results: 8/27 had low splenic volume, 9/27 had moderate splenic volumes and 10/27 had high volumes of spleen. When the nine subcategories were analyzed individually, 50% of children with low splenic volume was seen in < 8 years, all with high risk, majority had N-WNT/SHH subtype, with median WBC of 3.15 x 10 3/µl and OTT of 41 days whereas in 8–12 years it was 2.6 x 10 3/µL with OTT 59 days and in >12 years, 3.3 x 10 3/µL with OTT 47 days. All the patients had high risk and had CR to RT. Among children with moderate splenic volume, 66% were 8–12 years with a median WBC 2.6 x 10 3/µL, predominantly SHH and OTT 57 days. All High-risk Patients had PR. Children >12 years had median WBC count 2.7 x 10 3/µL and OTT 60 days. All high-risk patients showed partial response. Age <8 years had Median count: 2.6 x 10 3/µL and OTT 62 days. Response to RT were either stable or partial. Among high splenic volume children, 50% were >12 years, majority being non-SHH/non-WNT and median WBC of 3.37 x 10 3/µL and OTT 58 days and 20% recurred at 1 year. Patients <8 years, had a WBC of 3.1 x 10 3/µL and OTT 57 days. 50% had disease recurrence. Age 8–12 years showed progression after RT and death with a median WBC of 2.8 x 10 3/µL and OTT 59 days.

Conclusions: Splenic volume could be an indirect measure of compliance and response to RT. CR was seen in high-risk and < 8 years having low spleen volume. The WBC trend in children with moderate splenic volumes caused longer overall treatment time. Children with high spleen volumes had poor response to RT and higher deaths among all age groups.

Recurrent glioblastomas: A “Tartarean Abyss”

Himanshi Diwan, Anila Sharma, Garima Durga, Munish Gairola, IC Premsagar; Rajiv Gandhi Cancer Institute, Delhi, India

Introduction: Glioblastomas are the most aggressive brain tumors with a grim prognosis, accounting for 45%–50% of all primary malignant brain tumors, the prognosis being dismal in cases of recurrent glioblastomas. The current guidelines favor concurrent temozolomide and radiotherapy followed by maintenance chemotherapy. Despite advances in modern therapy, average survival is 12-18 months in spite of the known targets but lacking a targeted therapy.

Objective: This study is aimed at studying clinical and biomarker profile of recurrent glioblastomas in a tertiary care center.

Materials and Methods: We retrospectively studied all recurrent glioblastomas between the period of 2016–2020. The detailed biomarker testing for cases of glioblastomas at the time of first surgery and recurrence both was available in few cases.

Results: 15 cases of recurrent gliomas were analyzed comprising of 10 primary and 5 secondary glioblastomas with mean age of 49 years. All the primary glioblastomas showed wild-type IDH1(R-132-H) expression with retained ATRX and mutant p53. Of the 5 secondary glioblastomas studied, 3 had IDH1 mutant expression with loss of ATRX while 2 were IDH1 wild type with retained expression of ATRX. MGMT promoter methylation status was available in just 3 cases and 1 showed methylator phenotype. Mean interval of recurrence was 10–13 months with 2 cases recurring as early as 1.5 months.

Conclusion: All the primary glioblastomas that recurred had IDH1 wild type and retained ATRX profile and 90% had p53 mutation inferring that IDH1/ATRX/p53 mutation portends a poorer prognosis vis a vis IDH1 mutated glioblastomas with loss of ATRX. With the new WHO classification in the horizon, a detailed molecular profiling of such tumors may become possible and will help in further highlighting the biology of recurrent gliomas/glioblastomas thus paving a path for future targeted therapies.

Clinicopathological study of metastatic brain tumors in a cancer institute and research center

Vidya Menon, Anila Sharma, Gurudutt Gupta, Ullas Batra, D.C. Doval; Rajiv Gandhi Cancer Institute and Research Center

Background: Metastatic brain tumors, being the most common brain tumors in adults, are a frequent cause of morbidity and mortality among cancer patients. Brain metastasis is a grave incident having a huge impact on the survival of cancer patients. In patients with systemic malignancies, brain metastases occur in 10%–30% of adults and 6%–10% of children. The incidence of brain metastases is underestimated and is increasing due to both improved detection and better control of systemic cancer using advanced therapeutic techniques.

Objectives: This study aimed to evaluate the importance of identifying the primary site of tumors metastatic to the brain, to differentiate them from primary brain tumors as well as to summarize the diagnostic algorithm for doing so, using immunohistochemistry.

Materials and Methods: This study was conducted in the department of pathology of a cancer institute by retrospectively collecting data of patients with metastatic brain tumors over a 5-year duration from January 2015 to December 2020. A total of 55 patients diagnosed with brain metastases during these five years were analyzed with respect to the wide array of immunohistochemical markers used to identify the primary site of cancer thus assisting in providing targeted therapy. The characteristics of brain metastatic tumors from different primaries were studied with respect to their relative incidences, differing initial presentations, clinical symptoms, the number of brain lesions at diagnosis, age at presentation, and overall survival and treatment.

Results: Lung cancers were the most common tumors to metastasize to the brain followed by breast cancer. The other primary sites included female genital tract, prostate, gastrointestinal tract, salivary gland and soft tissue sarcomas in that order. Breast cancer was the most frequent cancer to present with brain metastasis at an early age. Majority of the patients with brain metastases were treated with surgery followed by postoperative radiotherapy and targeted chemotherapy. Majority of brain metastases presented as multiple lesions at initial diagnosis on magnetic resonance imaging and positron emission tomography scans.

Conclusions: An immunohistochemical algorithmic approach is the key to evaluate brain metastases in an unknown primary, or to confirm the primary site in a known case of systemic malignancy, or to differentiate these from primary brain tumors. This helps in guiding targeted therapy for the particular cancer and aids in better patient management and improved survival.

Dura-based recurrence of epithelioid glioblastoma in a young adult: A case report

Ramesh S; Jubilee Mission Medical College and Research Institute

A 24-year-old male presented with intractable seizures due to a right frontal premotor lesion for which he underwent surgery in early 2019 with the biopsy reported as Pilocytic astrocytoma. He had a recurrence of tumor with the imaging showing a large right frontal lesion with intratumoral bleed. The tumor was excised and the biopsy showed that he harbored a glioblastoma now, possible epithelioid variant with IDH1, BRAF, and MGMT negative. He underwent adjuvant radiotherapy and treatment with Temozolomide. Follow-up scan at 3 months showed no significant recurrence, but at 5 months showed recurrence along the right temporal basal dura and the tentorium, with intratumoral and subdural bleed. Tumor was excised and the biopsy reported as glioblastoma. This case is being presented because of the rarity of epithelioid glioblastoma and also the dural recurrence.

A clinicopathological study of inflammatory pachymeningitis: Emphasis on IgG4-related disease of the dura

Rima S, K Prabhu, Chacko AG, Ranjith K Moorthy, Joseph BV, Mathew J, Chacko G; Christian Medical College

Introduction: IgG4-related disease is an immune-mediated fibro-inflammatory condition. Central nervous system involvement was initially described as a form of hypophysitis and later described in a subset of patients with idiopathic hypertrophic pachymeningitis. IgG4 related disease is not an “uncommon entity,” but it is often misdiagnosed or under recognized.

Objectives: To assess the clinical, biochemical and pathological parameters of IgG4 related diseases of dura in cases with a clinical diagnosis of idiopathic hypertrophic pachymeningitis.

Materials and Methods: Cases with a diagnosis of hypertrophic pachymeningitis were retrieved from the archives of the Pathology Department from 2010-2020. The hematoxylin and eosin slides and immunohistochemical stains for IgG and IgG4 were reviewed. The clinical presentation, serum levels of IgG and IgG4, radiological findings, treatment and the clinical behavior of the disease were also studied.

Results: A total of 20 cases were identified with a diagnosis of pachymeningitis. The age of presentation ranged from 20 to 61 years (mean 41.3 years) with an equal gender distribution. The clinical presentations were of multiple cranial nerve palsies (40%), headache (25%) and seizures (15%). The radiological appearances included extra-axial mass (37%) and dural thickening(26%). A moderate to marked plasma cell-rich chronic inflammatory infiltrate as seen in 18 out of 20 cases (90%). Fibrosis was seen in most of the cases, which was angiocentric in two of the cases. There were no cases with storiform fibrosis or obliterative phlebitis. Special stains for microbial organisms were negative in all the cases.

Serum IgG4 values were available for 11 out of 20 patients (55%) and was elevated in 7 cases, of which only one had a IgG4

IgG positive plasma cells of more than 40%.A significant ratio of IgG4

IgG was seen in 4/18, of which two showed emperipolesis and were diagnosed as Rosai-Dorfmann disease. These patients responded to treatment with glucocorticoids––Mycophenolate or Azathioprine––were added on as second-line agents.

Conclusion: Although all cases in this series had plasma cell-rich inflammatory infiltrates, only 20% had a significant IgG4

IgG ratio of which two showed histological features of Rosai–Dorfmann disease. Storiform fibrosis and obliterative phlebitis do not seem to be features of hypertrophic pachymeningitis. This suggests that the IgG4 disease of the dura may have a spectrum of histological features that differ from other organ systems and larger studies are required to characterize this disease further.

Cyst wall dynamics during and after imageguided pencil beam scanning proton therapy in craniopharyngiomas

Nagarjuna Burela, Srinivas Chilukuri, Rajesh Thiyagarajan, Noufal MP, Adithyan R, Dayananda Sharma S, Rakesh Jalali; Apollo Proton Cancer Center

Purpose: Craniopharyngioma cyst wall changes potentially perturb pencil beam scanning proton therapy (PBS-PBT) dose distributions. This study evaluates cyst wall dynamics and its dosimetric impact during the PBS-PBT.

Materials and Methods: Eleven consecutive patients with craniopharyngioma treated with image-guided PBS-PBT were analyzed. PBS-PBT plans were generated after target delineation to dose of 54CGyE/30fractions with robustness up to 3mm setup errors and +3.5% range uncertainty. Weekly quality assurance (QA) CT and/or MRI scans were performed to assess the cyst wall dynamics and PBT dose perturbations.

Results: Total of 34 QA scans were performed with median of 3 scans per patient. Four patients (36%) showed cyst deformation. Three patients showed an increase in the volume (median 0.52cc; range 0.15–1.16cc) and one showed a reduction in the volume (0.8cc). Largest changes to the cyst wall volumes were noted after 3 weeks in 2 patients and at 4 weeks in 2 patients. None required adaptive re-planning as no significant dose perturbation to GTV was noted. Median PTV dose perturbation was 0.32% (range 0.16 to 0.8%). Mean percentage change from the nominal plan for temporal lobes, hippocampus and optic chiasma were 1.2%, 1.3%, and 0.5%, respectively. With a median follow-up of 14 months (3–24 months), 5 patients showed shrinkage of residual tumor/cyst and rest showed stable disease.

Conclusion: We have demonstrated craniopharyngioma cyst wall deformations during PBS-PBT in more than one-third of our patient cohort without significant dose perturbations to targets and OARs, reinforcing a need for stringent QA imaging during treatment of these patients.

Multifocal pediatric anaplastic astrocytoma: A case study

Pallav Prasun, Manish Gupta, Vipin Kharade, Saikat Das; AIIMS, Bhopal, Madhya Pradesh, India

Pediatric high-grade gliomas, including glioblastoma and anaplastic astrocytoma, make up 8%–12% of pediatric central nervous system tumors and have poor prognosis, with 2-year survival less than 30% and overall survival less than 10%. The role of temozolomide, an alkylating agent, in pediatric HGG is not well understood. We present a case of multifocal anaplastic astrocytoma in a 12-year-old girl managed with subtotal resection followed by adjuvant whole-brain irradiation and concurrent temozolomide followed by adjuvant temozolomide with continued survival over 18 months.

The girl belonging to lower socio-economic strata visited our OPD in 3rd week of November 2019 for post-operative management of a high-grade glioma (WHO Grade III). She had suffered from headache and episodes of vomiting for 3 months which were not attended to. It was after she developed left-sided hemiparesis a month later that her family sought medical attention. There was slurring of speech associated. However, there was no history of seizures, neck rigidity, fever or signs and symptoms of neurocutaneous syndromes. Contrast-enhanced MRI of Brain at a tertiary care government hospital revealed -1. Multifocal multicystic septated superficial cortical based lesion in bilateral occipital, right temporo-parietal and left temporal lobe with few areas of GRE blooming in left temporal lobe; 2. A focal 4.4X6.2 cm large T1 hypointense and T2 hyperintense unilocular cystic lesion in right parieto-temporal region causing mass effect (midline shift of 7.7 mm to left) and effacement of ipsilateral lateral ventricle; 3. A poorly circumscribed 4.0 cm × 4.2 cm T2 and FLAIR hyperintense lesion in left parietal lesion. She underwent right FTP craniotomy with subtotal resection and decompression of tumor. Surgical Pathology revealed features suggestive of high-grade glioma which was later reviewed at our center which further specified it to be a WHO Grade III Glioma. Her condition improved post-surgery only to deteriorate further which prompted her to visit AIIMS Bhopal for further management. After assessment and exploring various options at multidisciplinary meeting, it was decided to offer our patient Adjuvant RT in two phases along with oral Temozolomide.

Our case study helps explore the prospect of adding temozolomide to the armamentarium of managing pediatric high-grade gliomas which is lethal in children across all ages. Ongoing advancements in molecular characterizations may further enhance our understanding of nature of HGGs in pediatric population and improve management of the same.

Dosimetric comparison of 3D conformal radiotherapy and volumetric modulated arc radiotherapy in craniospinal irradiation for pediatric radiotherapy in germ cell tumor

Arnav Tiwari, Saikat Das, Anju KV, Manish Gupta, Vipin Kharade; AIIMS, Bhopal, Madhya Pradesh, India

Objective: Optimization of technique of craniospinal irradiation (CSI) is a challenge in pediatric radiotherapy. Diseases like Germ Cell tumor which has good therapeutic outcome, improved dosimetric plan becomes relevant due to long term implication in quality of life. A single field treatment is not usually possible due to field length. More than one isocenter and inhomogeneous dose distribution specially at brain and spine junctions pose challenges in optimization where single gradient techniques are relevant. This study is a dosimetric comparison between 3D conformal radiotherapy(3DCRT) and volumetric modulated arc radiotherapy (VMAT) in pediatric CSI.

Materials and Methods: A dosimetric comparison was undertaken for a germ cell tumor patient candidate plan for CSI. Patient was simulated in supine position. For brain PTV a 360⁰ arc VMAT was compared with conventional right lateral and left lateral beam plan. For Spinal PTV a 50⁰ arc VMAT plan was compared with single spine to spine junction traditional PA field. Feathering technique was used to prevent over/under-dosing at junctions. The resultant planed doses were compared for homogeneity, junctional doses, hot and cold spot volumes, OAR doses, and monitor units.

Result: Comparison showed a better dose conformity (0.915 vs. 0.946), homogeneity (1.16 vs 1.10) and higher PTV coverage (92% vs. 96%) with VMAT plan. Volumes of hot spots and cold spots within PTV were low in VMAT. Monitor units for VMAT were twice as that of 3DCRT. The result is shown in [table 1] below:{Table 1}

Conclusion: Both, optimized 3DCRT and VMAT, can achieve satisfactory dosimetry for craniospinal irradiation in pediatric radiotherapy. However, from this single case it appears VMAT has advantage in PTV coverage over 3DCRT. Other OAR coverage was comparable. Further dosimetry studies on larger number of patients are needed keeping the radiotherapy facility throughput, machine load and other treatment-related factors in consideration.

Synchronous central nervous system nongerminomatous germ cell tumor: A case report

Divya Taragi, Manish Gupta, Saikat Das, Vipin Kharade; AIIMS, Bhopal, Madhya Pradesh, India

Background: Central nervous system germ cell tumors (GCT) are rare brain tumors occurring in adolescents and young adults. Intracranial GCT includes germinomas and non-germinomatous tumors. They often occur in the midline region, with a higher percentage in the pineal region and others in the suprasellar region. Synchronous tumors in the pineal and suprasellar region are a rare entity seen in GCTs and are empirically diagnosed as germinomas. We describe a case of a 16-year-old male with a secretory synchronous non-germinomatous germ cell tumor treated with chemotherapy followed by craniospinal irradiation.

Case: A 16-year-old male presented with complaints of headache associated with vomiting and diplopia. MRI Brain revealed an intra-ventricular mass lesion in the right lateral ventricle with midline crossing and associated hydrocephalus and lesions in pineal gland and pons which was suggestive of neoplastic mass lesion–– possibly germinomas. His serum alpha-fetoprotein (AFP) and human chorionic gonadotropin levels were 17896 IU/mL and 0.759 mIU/mL, respectively, were in favor of non-germinomatous germ cell tumor (yolk sac tumor). Biopsy could not be performed because of the risk involved as was discussed in the tumor board. Cerebrospinal fluid (CSF) cytology was deferred due to raised intracranial pressure. He received 2 courses of high-dose chemotherapy with Carboplatin, Ifosfamide, and Etoposide. MRI spine showed no significant abnormality. CSF examination done post-chemotherapy showed few lymphocytes and occasional polymorphs. The CSF AFP level was normal. He was then planned for craniospinal irradiation which was given over 6 weeks using volumetric modulated arc therapy. The follow-up AFP levels were normal and the patient has been doing well for 6 months.

Discussion: Synchronous GCTs are often diagnosed as germinomas as the literature to support the diagnosis of non-germinomatous germ cell tumors is essentially non-existent. It is imperative to consider non-germinomatous tumors in the cases of synchronous lesions as the treatment as well as the prognosis for germinomas and non-germinomas varies drastically. A biopsy is advisable wherever possible while considering the high risk of bleeding associated with GCTs. Our patient was diagnosed with a non-germinomatous germ cell tumor as the AFP levels were high and HCG levels were within the normal range, favoring the yolk sac tumor. He was then treated with high-dose chemotherapy and craniospinal irradiation and has been doing well for 6 months.

Is hypofractionated stereotactic radiotherapy (hfSRT) in intracranial arteriovenous malformations (AVMs) still an option?

Swathi B, Rajesh B, Raj Krishna B, Patricia S, Selvamani B; Christian Medical College, Vellore, Tamil Nadu, India

Objective: To analyze the outcome of hypofractionated stereotactic radiotherapy in AVMs.

Materials and Methods: Patients who had large volume AVMs (>3cm, >25 cc), SM grade 3 or above and lesions that were close to critical organs were planned with hypofractionated SRT after discussion in the multidisciplinary board. Forty-eight patients who were treated with hfSRT from September 2008 to December 2019 and who had at least one follow-up after completion of hfSRT were identified from our database. Clinical and radiotherapy details were retrieved retrospectively from electronic medical records and BrainScan /iplan databases. We used Gill Thomas Cosman (GTC) frame for immobilization in all patients. Contouring was done by fusion of DSA images and planning scan. The AVMs were treated to a dose of 30–36 Gy in 5-6 fractions on alternate days over a period of 2 weeks.

Results: Thirty 1% of patients had AVMs in parietal lobe followed by frontal region (18%). Mean target volume was 34.07 cc. Median follow-up period was 18 months (range 7–85 months). On follow-up, 4 patients had complete obliteration, 24 patients had partial obliteration, 13 had stable disease and 2 had disease progression. Two patients had radiation necrosis, two had intracranial hemorrhage and one patient had death not related to AVM. Symptomatic improvement was seen in neurological status in 56% patients, Seizure control in 45% and reduction in headache in 69% of patients.

Conclusion: Hypo fractionated SRT is a safe and feasible option in large AVMs which are not amenable for SRS/ embolization/ surgery.

Differential expression of glial mesenchymal transition markers in cultured primary glioma cells

  Venkatesh, Manas Panigrahi, Y B V K Chandrashekar, Beevi SS,   Shailja; Kims Hospital Hyderabad Telangana

Background: Median survival time in glioma varies with grade, in high-grade glioma it is 15–16 months, in low-grade it is 5–7 years. Low-grade gliomas eventually progress to high-grade glioma

Aim of Study: To compare proliferative index and its implication in survival of different grades of glioma patients, and to study novel markers in glioma

Materials and Methods: Glioma tissue from 45 patients were collected after Ethics approval and cultured as primary cells in DMEM/F12 medium supplemented with Fetal bovine, EGF and FGF. Based on the Population doubling time, cultured cells were categorized as high proliferation rate (having PDT < 72 h), low proliferation rate (having PDT > 72h). All the cultured cells were analyzed for the presence of GFAP, vimentin, N-cadherin, TWIST and cMyc. Cell viability was measured after 48-h treatment with the indicated concentration of temozolomide

Results: Different pattern of proliferation irrespective of their grade and IDH status was observed in cells. Progression-free survival and overall survival period compared in each group according to proliferation index. In low prolifrerative cultures overall survival period and progression-free survival period is high when compared to high proliferative index cultures. Low proliferating cultures of grade II, III and IV tend to be more sensitive to TMZ when compared with high proliferating cultures. HPI correlating with strong expression of GMT markers as compared to LPI group.

Conclusion: 1. There is a progression-free survival difference according to proliferative index in grade 2 and grade 3 patients. There is an overall survival difference in grade 4 patients according to proliferative index, high in low proliferative cultures and low in high proliferative index

2. Low proliferating cultures of grade II, III, and IV Glioma tend to be more sensitive to TMZ when compared with high proliferating cultures

3. HPI correlating with strong expression of GMT markers as compared to LPI group.

A rare case report of meningomyelocele with epidermoid cyst

Christopher Zodinsanga Pachuau, K Balakrishnan; PGT at KAPV Govt Medical College

Introduction: Spinal epidermoid cyst is a benign lesion composed of stratified squamous epithelium. Congenital spinal epidermoid cyst results from anomalous implantation of ectodermal cells during a closure of the neural tube between 3rd and 5th week of embryonic life.

Clinical History: A male baby 28 days old was brought by his mother with midline swelling in lower back. The patient was born with hydrocephalus and meningomyelocele. Family history of third-degree consanguineous marriage was elicited. Surgical excision and repair were done.

Materials and Methods: Specimen received in 10% formalin.

On gross examination, we receive a membrane soft tissue (already cut opened) measuring 3 cm x 2 cm x 0.5cm. No solid area. All Embedded.

Results: Microscopic examination shows a polypoidal lesion covered by epidermis containing bland neural tissue with scattered pigmented cells and adnexal structures. There is an associated dilated cyst with corrugated flattened epithelial cells and keratin material. No atypia.

The diagnosis is given as meningomyelocele with epidermoid cyst.

Molecular landscape of teenager and young adults with medulloblastoma

Anbarasu Lourdusamy, Maria Estevez Cebrero, Omar Ahmad, Andrew Peet, Richard Grundy, Anbarasu Lourdusamy; University of Nottingham

Objectives: Medulloblastoma, a malignant brain tumor occurs mainly during childhood but is also observed in the teenager and young adult (TYA) population frequently enough to warrant attention. Considering that medulloblastoma (MB) is a biologically and clinically heterogeneous tumor with four molecular subgroups - Wingless (WNT), Sonic Hedgehog (SHH), Group3 and Group4, this study aimed to define the molecular landscape of MBs in TYA.

Materials and Methods: We collated all MB samples with transcriptomics data from the five published cohorts (n = 1234), including 181 MB samples from TYA patients age between 13 and 24 years. Using computational approaches, we estimated relative fractions of diverse immune cell subsets in gene expression profiles of TYA MBs.

Results: MBs in TYA comprised of heterogeneous molecular groups, with the predominant representation of Group4 (50%) and SHH (29%), but also WNT (13%) and Group3 (8%) subgroups. In the TYA population, a large fraction of Natural Killer T (NKT) cells was present in Group3 (0.35 ± 0.14), Group4 (0.25 ± 0.11), SHH (0.21 ± 0.13), and WNT (0.19 ± 0.09) tumors. Surprisingly, the M2 immunosuppressive macrophages were low in SHH (0.09 ± 0.06) when compared with WNT (0.18 ± 0.08) and Group 3 (0.12 ± 0.08). Compared to pediatric (less than 13 years) and adult (24+ years) age groups, 241 genes were significantly differentially expressed in the TYA group of SHH subgroup, whereas no gene was detected in the Group4 subgroup (False discovery rate, FDR < 0.05). The majority of 241 genes showed high expression in TYA (84%) and were significantly enriched for microtubule-associated genes including dynein and tubulin. Besides, several homeobox genes showed high expression in TYA when compared with children and adults in SHH.

Conclusions: In MB, TYA patients were enriched in Group4 and SHH along with relatively low Group3 tumors. Our study also demonstrated the molecular landscape of TYA and delineated the immune component of the MB tumor microenvironment.

Sellar chondroid chordoma

Ojaswi Vijay Garg, Maneesh Kumar Vijay, Neha Sethi, Disha Hassani, Akanksha Thatai, Manju Raghava; Mahatma Gandhi Medical College and Hospital, Jaipur, Rajasthan, India

Introduction: Chordoma is a rare malignant tumor with notochordal differentiation. It accounts for only 1% of all malignant bone tumors and in that only 0.1%–0.2% are intracranial. Chordoma involving the sella turcica is very rare. It usually occurs in adult female as compared to males.

Objectives: To study a rare case of chondroid chordoma in the sellar region, which is a very rare site for these tumors.

Materials and Methods: The present case was a 59-year-old female who presented with complaints of headache and left eye vision loss. Radiologically fungal lesion was suspected. She was operated from endoscopic endonasal trans-sphenoidal decompression of sphenoidal mass. Intraoperative findings showed a sellar mass that was white-yellowish with ill-defined borders, fungating, suckable, non-vascular and eroding sella turcica. Tissue was sent for histopathological examination.

Result: Histopathologically two possibilities were given-chondroid chordoma vs well-differentiated chondrosarcoma. IHC was done to reach the final diagnosis.

Conclusion: Sellar chordoma can be misdiagnosed as it clinicoradiologically mimics pituitary adenoma so it is important to know about it. As sellar chordoma is extremely rare, it should be included in the differential diagnosis of tumors occurring in the sellar region especially when it presents as a calcified tumor with bony erosion.

Keywords: Chondroid chordoma, immunohistochemistry, sellar mass

Opsoclonus myoclonus syndrome in neuroblastoma

Shreya Gandhi, Anshita Arora, Sujata Mushrif, Purna Kurkure, Sajid Qureshi, Shilpa Kulkarni, Anaita Udwadia Hegde; NH SRCC Hospital, Mumbai, Maharashtra, India

Introduction: Opsoclonus myoclonus ataxia (OMA) syndrome is a rare neuro-immunological disorder characterized by acute onset ataxia, opsoclonus, myoclonus, irritability, behavior and sleep disturbances and regression of milestones. Its onset is usually in first 2 years of life with paraneoplastic (neuroblastoma) and para-infectious (viral infections) causes being the most common etiologies.

Materials and Methods: A retrospective review of 20 children diagnosed with OMA syndrome over a decade at 3 tertiary care centers of Mumbai was done and analyzed. Disease outcome was assessed in terms of complete or partial resolution at 3months, 6 months and 1 year of treatment. Treatment options were monotherapy with ACTH or 2 or 3 drug polytherapy with ACTH, IV immunoglobulin (IVIg), rituximab or steroids.

Results: Female to male ratio was 2.3:1. The most common etiology was neuroblastoma (75%), representing the paraneoplastic group. Para-infectious and idiopathic etiologies were seen in 25% of patients. The most common clinical presentation was ataxia (100%), followed by opsoclonus (95%). Irritability and behavioral issues were seen in 75%. 60% of the patients had myoclonus and speech regression, respectively, and 45% had tremors. The mean duration of treatment was 17 months for neuroblastoma and 19.4 months overall. Monotherapy was used in 15% of the patients and polytherapy was used in 85% with 2 drug polytherapy in 60% and 3 drug polytherapy in 25%, respectively. Disease outcome at 3 months showed partial resolution in 75% and complete resolution in 25% patients. At 6month follow-up, 55% had partial resolution and 45% had complete resolution. And in 12month follow-up, partial and complete resolution was seen in 50% patients each. Of these, the patients receiving monotherapy showed 67% partial and 33% complete resolution, respectively, at 3 months, 6 months, and 12 months. Those receiving 2 drug poly therapy showed 75% partial and 25% complete resolution at 3 months, which gradually increased to 58.3% and 50% partial resolution at 6 and 12 months, respectively, and 41.6% and 50% complete resolution at 6 and 12 months, respectively. However, the most significant improvement was seen in those receiving 3 drug polytherapy. With the 3 monthly follow-up showing 80% partial and 20% complete resolution, which increased to 40% at 6 months and 33% partial resolution at 12 months and 60% and 67% complete resolution at 6 and 12 months, respectively.

Conclusion: OMA syndrome with neuroblastoma can be treated effectively if diagnosed early with initiation of aggressive therapy. Paraneoplastic etiology and polytherapy have better disease outcomes.

Curcumin at a lower dose is effective in reducing cytokine IL-6 and stem cell marker CD-133 expression in glioblastoma

Kavita Peter, Ragini Gothalwal, Puneet Gandhi; Barkatullah University, Bhopal, Madhya Pradesh, India

Objective: Glioblastoma (GB) is the most aggressive diffuse glioma of astrocytic lineage. The current multimodal therapy has minimally improved the median survival of patients from 12 to 14.6 months in GB. This poor survival is an outcome of many contributing factors the major being resistance due to the presence of a subset of cells called Glial stem cells (GSCs) which are triggered by chronic inflammation within the tumor and its microenvironment. Thus, there is an exigent need for exploring new non-toxic natural molecules that can mitigate resistance and relapse which holds the potential to improve brain tumor therapies. Herein, we investigated the anti-stemness and anti-inflammatory effect of curcumin on human GB cells at a very low dose. Curcumin is known to cross the blood-brain barrier and has presented remarkable safety in clinical trials.

Materials and Methods: U-87 GB tumor cells were cultured and treated with various concentrations of curcumin for 48hr. Cell viability was assessed by MTT assay. To detect whether curcumin abolished cell cycle progression, PI staining, and flow cytometry assay was performed in GB cells treated with 20 μM curcumin for 48 h. A clonogenic assay was performed to assess how many cells were able to retain clone forming capacity on treatment. The tumorigenicity of the cells was evaluated by soft agar colony formation assay, to determine the tumorigenic potential of malignant cells by evaluating their ability to survive in an anchorage-independent manner, a characteristic of GSCs. To study stem cell inhibitory effect of the curcumin, CD-133 tumor stem cell marker was analyzed in treated and untreated cell samples using flow cytometry. In order to decipher the anti-inflammatory potential of a low dose of curcumin, pro-inflammatory cytokine IL-6 was assessed by ELISA.

Results: A very low dose of curcumin (20 μM) decreased the cell growth and arrested the cells in the G2/M phase of the cell cycle. Curcumin also inhibited the anchorage-independent and anchorage-dependent clone forming capacity which is characteristic of GSCs. The treatment also reduced the expression of stem cell marker CD-133 and pro-inflammatory cytokine IL-6.

Conclusion: Taken together curcumin is able to exert anti-tumor action through impeding GSCs and lowering inflammation. Curcumin known for its non-toxicity and pleiotropic molecular targeting might be further explored as an adjunct with established brain tumor therapies particularly addressing resistance and thus positively influencing survival rates and quality of life of patients.

Clinical outcomes of ependymoma treated with radiation therapy: A single-institution experience

Jino Wilson, Rajesh B, Rajkrishna B, Patricia S, Selvamani B; Christian Medical College, Vellore, Tamil Nadu, India

Objective: To evaluate the clinical outcomes of ependymoma treated with surgery followed by radiation therapy in our institution.

Materials and Methods: We analyzed the electronic medical records and radiation details of 40 patients with Ependymoma who were treated with radiation at our institution from 2017-2019. The parameters documented were patient demographics, tumor characteristics, surgery and radiation treatment aspects, and outcomes.

Results: The age of the patients ranged from 1 to 50 years (median of 23 years) out of which 17(42%) were below the age of 16. Male-to-female ratio was 1.5:1. Common symptoms and signs at presentation were headache (62.5%), vomiting (55%), and neurological deficits (15%). All patients were evaluated with MRI brain with spine screening and 11 patients (27%) had CSF analysis prior to treatment. Thirteen patients (32.5%) had supratentorial tumors, 19 (47.5%) infratentorial tumors and 8 (20%) spinal tumors. Drop metastases to spine were seen in 4 patients (10%). Majority of patients (95%) underwent upfront surgery – gross/ near-total excision (65%); subtotal excision (20%); partial excision/biopsy (15%). All Grade 1 ependymomas were spinal tumors and Grade 2 and 3 ependymomas were located in brain. All patients received RT, either post-surgery or at recurrence. Children <2 years of age received chemotherapy followed by RT at a later date. Median dose of RT was 54Gy –supratentorial and infratentorial tumors received 54-59.4Gy and 50.4Gy in spinal tumors. The technique of RT was – conventional in 24 patients (60%), 3DCRT in 14 patients (35%) and VMAT in 2 patients (5%). Median duration of follow-up was 8.5 months (0-119 months). At follow-up, local recurrence was found in 6 patients (15%) and spine metastases in 2 patients (5%). Drop metastases to spine (P = 0.007) and Grade 3 tumors at diagnosis were significant risk factors for recurrence. At recurrence, 3 patients were treated with surgery followed by re-irradiation, 1 had surgery followed by chemotherapy and 4 had re-irradiation alone. The median disease-free survival for the entire cohort was 17 months (range: 3-84 months) and median overall survival was 22 months (3-115 months).

Conclusion: Surgery followed by radiation therapy is the standard of care for ependymomas. Our study showed a good local control rate of 80% with surgery followed by RT but requires long-term follow-up. Higher rates of recurrence were seen in patients with grade 3 tumors and patients with drop metastases at presentation.

Cerebellopontine angle epidermoid cyst: A series of 11 cases

Diljeet Kaur, Gajendrapal Singh Uike, Sudha Iyengar; Gajra Raja Medical College, Gwalior, Madhya Pradesh, India

Introduction: Epidermoid are uncommon lesions of central nervous system. These tumors are benign and constitute 0.2%–2.0% of all the intracranial tumors, for intracranial epidermoid––CP angle is the most common site. Other common sites are suprasellar region, diploic skull, floor of the fourth ventricle and spinal cord.

Materials and Methods: this is a retrospective study done in the department of pathology G.R.M.C. Gwalior. The study comprises of 11 cases in a span of one and half years.

Result: in our study out of 11 cases we found epidermoid cyst to be slightly more common in females (54.5%) than in males (45.5%). Majority of the cases were seen in the age group of 21-40 years.

Conclusion: Epidermoid of central nervous system is quite rare. We have a series of 11 patients in span of one and half years as our hospital is a premier institute of neurosciences in Madhya Pradesh.

In our setup we found the mean age was 28 years and more common in females than males.

Burden of childhood CNS tumors in radiotherapy department: A tertiary care center experience from India

Renu Madan, Aditya K Singla, Shikha Goyal, Divya Khosla, Sushanta K Sahoo, Ashish Aggarwal, KIrti Gupta, Debajyoti Chatterjee, Chirag K Ahuja, Amita Trehan; PGIMER, Chandigarh, India

Objective: Primary central nervous system (CNS) tumors are the most common solid tumors in children and young adults (CYA), leading to severe disabilities and high burden on families and health care facility. Clinical presentation and histopathological variants of CNS tumors in CYA differ significantly from adult counterpart. Since there is a paucity of reliable data on the epidemiology of these tumors in the developing world, we evaluated our data to understand the demography and histology of CNS tumors in CYA.

Materials and Methods: The data were prospectively maintained in the Microsoft Excel spreadsheets. CNS tumor patients of age group 0–18 years, registered in the department of Radiotherapy over a period of 18 months from April 1, 2019 to October 30, 2020 were analyzed for demographic and clinic-pathological details. Number of various histologies was calculated with respect to age and gender. Descriptive statistics was done using SPSS software, version 23.0.

Results: A total of 851 primary CNS tumor patients were registered in our department during the specified period. Out of this, 105 (12.3%) patients belonged to CYA age group. Children up to 15 years of the age accounted for major burden (n = 92, 87.6%). There was a male predominance with a male to female ratio of 1:6. Among all pediatric age groups, brain stem glioma was the commonest malignancy (n = 31) followed by medulloblastoma (n = 29), ependymoma (n = 16), grade 1 astrocytoma (n = 7), grade 2 and 3 astrocytoma (n = 6), optic nerve glioma (n = 4), glioblastoma (n = 4), craniopharyngioma (n = 3), germ cell tumors (n = 3) and embryonal tumors with multi-layered rossetts (n = 2). Among children aged 0-10 years, brain stem glioma was the commonest malignancy followed by medulloblastoma and ependymoma. However, medulloblastoma was on the top list in 11–15 years of age group followed by brain stem glioma and ependymoma. In contrast, astrocytoma was the most common tumor in older children aged 16 years or more. Fourteen patients (1.3%) required sedation/anesthesia for radiotherapy.

Conclusion: Radiotherapy plays an important role in the management of childhood CNS tumor patients. Thus assessment of patient load in radiotherapy department can improve our understanding of overall incidence of these tumors in India which may further help in resource development and prioritization.

Pediatric high-grade glioma: A diagnostically challenging case

Gaurav Khanna, Sunita Ahlawat, Rana Patir, Rakesh Gupta, Swati Mahajan, Vaishali Suri, M.C. Sharma; SRL Fortis Memorial Research Institute, Gurugram, Haryana, India

Background: Pediatric high-grade gliomas are a heterogeneous group of neoplasms with different molecular drivers. This is a case with unusual histological, immunohistochemical and molecular features.

Objective: Molecular characterization of a tumor showing features of pleomorphic xanthoastrocytoma and gliosarcoma with rhabdomyoblastic differentiation.

Materials and Methods: A 14-year-old female patient presented in our institute FMRI with history of three episodes of seizures since 2016 and intermittent headache and vomiting for past 2 years. Her visual field analysis revealed significant field defects. There were no other motor/sensory deficits. On MRI there was a well-defined lesion involving left temporo-occipital lobe and extending into the parietal lobe. Dural attachment, areas of cystic change and necrosis was also noted. Left occipital craniotomy and tumor excision was done. Intraoperatively It was a moderately vascular solid tumor with distinct margins from surrounding brain and attached to dura.

Provisional diagnosis of Meningioma and differential of astrocytoma was kept and tissue was sent for frozen which showed features of pleomorphic xanthoastrocytoma. No high-grade features were noted in frozen section evaluation. Tumor was received in multiple fragments altogether measuring 11x7x3.5cm. Entire tissue was submitted for processing.

Results: Tumor showed large pleomorphic cells having prominent nucleoli, brisk mitosis and scattered lymphocytes. Other areas showed spindle cell morphology which also displayed high-grade nuclear atypia, brisk mitosis and high ki 67 proliferative index in both the glial and spindled areas. Tumor expressed GFAP, S100, Olig 2 and CD 34. However, it was negative for EMA, PR and SOX 10. Interspersed small round undifferentiated cells were positive for Desmin and Myo D1 while negative for SMA. IDH and ATRX were non-mutated. Expression of INI1 was retained. P53 expression was wild type. H3.3G34R/V and H3k27M both were negative. However, BRAFV600E showed positive expression. FISH for CDKN2A showed homozygous deletion but EGFR amplification and PTEN loss was not detected.

Diagnosis of Diffuse pediatric type high-grade glioma, H3 wild type, IDH -1wild type with BRAV600E mutation and CDKN2A homozygous deletion was rendered.

Conclusion: A rare case of pediatric high-grade glioma with deregulation of MAPK pathway is highlighted. Awareness about the entity is crucial owing to significantly better overall survival compared to glioblastoma and availability of targeted therapy.

Acknowledgement: Department of Neuropathology, AIIMS New Delhi.

Anaplastic ganglioglioma: Description of a relatively rare entity

Reman R Nair, Geeta Chacko, J Ranjani, Baylis Vivek Joseph; Christian Medical College, Vellore, Tamil Nadu, India

Introduction and objective: Gangliogliomas (GG) are rare mixed glio-neuronal tumors seen in children and young adults, and are graded as WHO grade I and WHO grade III (anaplastic) tumors. Anaplastic gangliogliomas (AGG) represent 1%–5% of all GG and are associated with significantly worse local control rates and a 5-year survival of only 25%. We describe the clinicopathological features of three case of Anaplastic GG.

Materials and Methods: We retrospectively reviewed our medical records from 2004 to 2021 and detected three cases with a diagnosis of AGG. The clinical, radiological and histopathological features of these cases were studied.

Results: All three tumors were seen in young women mean age of 30 years, who presented only with headaches and no seizures. They were found to have papilledema on examination, but no focal neurological deficits. The first patient was seen in the year 2019 and had a 4.5 cm sized, contrast-enhancing tumor in the left temporal lobe with radiological evidence of hemorrhage. The second patient was seen in the year 2020, had a 5.6 cm right temporal mass, which was non-enhancing, and appeared to be a low-grade glioma. The third patient was seen in the year 2021 and was detected to have a 2.5 cm non-enhancing tumor, within the frontal horn of the left lateral ventricle, obstructing the left Foramen of Munro causing moderate hydrocephalus. All three patients underwent radical excision of the tumor. A histopathological diagnosis of anaplastic ganglioglioma was made on all three cases based on the presence of both neoplastic glial and neuronal components and high-grade features of mitotic activity, necrosis and microvascular proliferation. The first patient was given postoperative radiotherapy and temozolomide chemotherapy, and was disease free at 18-month follow-up. The second and third patients had an uneventful postoperative recovery, and are awaiting further adjuvant therapy.

Conclusion: AGG are rare neoplasms. Their high-grade nature can be difficult to diagnose radiologically. The diagnosis is made on histopathology. In our cases the tumors could be excised radically and the first case did well with postoperative radiation and chemotherapy. Larger studies need to be done to ascertain if this tumor entity has a better outcome compared to its astrocytic counterparts.

Immunophenotypic subtypes of primary central nervous system diffuse large B-cell lymphoma in Indian population

Ankur Kumar, Anila Sharma, Sunil Pasricha, Ramandeep Singh Jaggi, Anurag Mehta; Rajiv Gandhi Cancer Institute and Research Center, New Delhi, India

Introduction: Primary diffuse large B-cell lymphoma (DLBCL) of Central nervous system (CNS) is an uncommon neoplasm, accounting for 2%–3% of all brain tumors and <1% of all non-Hodgkin lymphoma. These tumors have a dismal prognosis and poor overall survival.

Objective: This study was aimed at determining the immunophenotypic subtype of Primary CNS DLBCL (PCNS DLBCL) in Indian patients, presenting at a tertiary healthcare cancer center.

Materials and Methods: A 6-year retrospective analysis (2015–2020) was done, for histologically diagnosed cases of CNS lymphoma presenting at our center. All relevant clinical and demographic details were obtained from medical records department. Those cases wherein paraffin blocks were available were subjected to extended immunophenotyping. These cases were classified and subtyped using Hans algorithm.

Results: Of 32 cases diagnosed as DLBCL with CNS involvement, 25 cases fulfilled the criteria of PCNS DLBCL in view of no clinical or radiological systemic involvement at the time of diagnosis. The majority of the PCNS DLBCL were seen in males (64%). The median age at diagnosis was 62 years. All cases of PCNS DLBCL were immunocompetent. Out of the 25 cases of PCNS DLBCL, extended immunophenotyping was possible for 19 cases. Using the Hans algorithm, 63% (12 out of 19) cases of PCNS DLBCL were classified as Non-GC like immunophenotype. Single case showed features of corticoid-mitigated lymphoma.

Conclusion: According to our study, majority of PCNS DLBCL, in the Indian population are of the Non-GC subtype. PCNS DLBCL has poor prognosis with overall 5-year survival of approximately 30%. Unlike the systemic DLBCL, where prognostic significance is well established, with non-GCB subtypes having a poorer prognosis, studies have failed to show any statistically significant difference, in terms of overall and disease-free survival between the two subtypes of PCNS DLBCL. This reflects a distinct clinical behavior compared to its systemic counterpart, suggesting the role of other factors, like, age, performance status, CSF protein, LDH level and involvement of deep brain structures, as highlighted by the International Extranodal Lymphoma Study Group.

Cryptococcomas mimicking a soft tissue tumor with multiple brain metastases

J Ranjani, Geeta Chacko, Rima S, Anne Jennifer Prabhu, Mohammed Shakeel Sillat, Ari G Chacko, Edmond Jonathan G; Christian Medical College, Vellore, Tamil Nadu, India

Introduction: Cryptococcomas are a rare complication of infection by invasive fungi of the Cryptococcus genus, forming a discrete lesion composed of immune infiltrates and pathogenic forms. The vast majority of infected individuals are immunocomprised. We report a case of multiple cryptococcomas involving soft tissue and central nervous system in a patient with poorly controlled diabetes mellitus type II.

Case Report: A 32-year-old male presented with a history of progressively increasing right calf swelling followed by left-sided faciobrachial weakness and headache for 6months. He was a newly diagnosed poorly controlled diabetic (DM) with an HbA1C of 9.6%. Ultrasound of the calf showed a well-defined hypoechoic lesion in the medial aspect of right proximal leg overlying the calf muscles in deep subcutaneous/deep fascial plane. MRI brain showed three separate multi-loculated supra-tentorial cystic lesions in the right cerebral hemisphere. The lack of perilesional edema and insignificant midline shift was notable considering the size of the lesions. We considered a multicentric glioma or cerebral metastasis from a probable primary tumor in the right calf. Fine needle aspiration and a core biopsy of the calf swelling failed to yield a result. After admission, his sensorium dropped and an emergency right fronto-temporo-parietal craniotomy and excision of the lesions were done. Postoperatively there was a significant improvement of the headache and faciobrachial weakness.

Histopathological examination showed cerebral parenchyma with a largely necrotic multicystic cavitary lesion with numerous round capsulated yeast forms ranging from 8-10 microns, resembling Cryptococcus neoformans. A larger biopsy of the calf swelling was done which showed a similar histology and the organism was confirmed with subsequent fungal isolation on culture. On reviewing the fine needle aspiration cytology smears several round to oval capsulated yeast forms, resembling Cryptococcus neoformans were identified.

Serum and CSF cryptococcal antigens were positive. The diagnosis of disseminated cryptococcal disease was confirmed based on Cryptococcus grown in cultures from lesions in the right calf and brain. Treatment was started with parenteral Amphotericin B for 4 weeks and flucytosine for 8 weeks.

Conclusion: Cryptococcomas can be easily mistaken for neoplasms in an otherwise immunocompetent patient and hence DM should also be considered a risk factor. Early and prompt recognition and treatment are advisable to prevent significant morbidity and mortality.

Molecularly aggressive ependymomas treated with Image-guided pencil beam proton therapy: Consecutive patient Indian experience

Rishan T S, Srinivas Chilukuri, Nagarjuna Burela, Utpal Gaikwad, Sham Sundar, Rajesh Thiyagarajan, Noufal Manthala Padannayil, Adhithyan R, Pankaj Kumar Panda, Dayananda Sharma, Roopesh Kumar, Rakesh Jalali; Apollo Proton Cancer Center

Aim: To assess the toxicities and early clinical outcomes in patients of primary and recurrent ependymomas treated with image-guided pencil beam scanning proton beam therapy (PBS-PBT).

Materials and Methods: Between January 2019 and 2021, we analyzed consecutive patients of ependymomas treated with image-guided PBS-PBT. They were also analyzed molecularly, and all recurrent/re-irradiated patients were considered for craniospinal irradiation (CSI). Acute toxicities were assessed based on NCI CTC v5.0, local control and radiological response by 3-monthly MRI post-therapy.

Results: Fifteen consecutive patients with ependymoma (11 Grade III and 4 Grade II) (median age 9 years) were analyzed. Seven were treated at first recurrence and 1 at second recurrence. Majority had posterior fossa (PF) tumors (11) followed by supratentorial (ST) (2) and spine (2). Among PF ependymomas, 9 had global loss of H3K27me3, whereas among ST, 1 had YAP1 fusion and other, negative L1CAM and p65/RELA on immunohistochemistry. Gross/near-total resection was achieved in 87% patients. Among 8 patients treated for recurrence, 7 received CSI followed by primary site boost to a total median dose of 55CGE (50.2-55.8CGE). Grade 2 dermatitis, grade 2 and 3 hematological toxicities (CSI) were noted in 3,2 and 2 patients, respectively. Grade 2 fatigue noted in 53% of all and 71% receiving CSI. With a median follow-up of 10 months (2-24 months), local control was 73% (upfront-100%, recurrent-50%).

Conclusion: Our experience of treating patients of ependymoma with complete resection followed by PBS-PBT including CSI is encouraging with low acute toxicities. Recurrent/molecularly aggressive cases may be considered for CSI.

Extraneural metastases from primary central nervous system tumors: A case series

Rajkrishna B, Rajesh B, Patricia S, Selvamani B; Christian Medical College, Vellore, Tamil Nadu, India

Introduction: Extraneural metastasis from primary central nervous system (CNS) tumors is rare. The incidence of extraneural metastases from Medulloblastoma ranges from 1-2%, Glioblastoma ranges from 0.4-0.5% and from Meningioma the incidence is less than 1%. However intracranial solitary fibrous tumor has a higher incidence of extraneural metastases ranging from 14% to 50%. The sites of metastases include bone, lungs and lymph nodes. We present 5 patients of primary CNS tumors with extraneural metastases treated at our center.

Aim: To evaluate the treatment outcome of patients with extraneural metastases from primary CNS tumors.

Materials and Methods: A retrospective review of five patients was conducted from 2014 to 2020. We present the demographic details, surgical, histopathological, radiotherapy details, management of metastases and outcome. Follow-up period was calculated from date of primary diagnosis. Spinal drop metastases were excluded from the study.

Results: There were 5 patients with extraneural metastases. The median age was 33 years (range: 31–57 years). Two were diagnosed to have intracranial solitary fibrous tumor, WHO grade 3 and one each with diagnosis of Glioblastoma, Medulloblastoma and Atypical meningioma, WHO grade 2.

Three had synchronous metastases - One with Atypical meningioma, one with Solitary fibrous tumor and one with Medulloblastoma. Atypical meningioma patient underwent near-total resection (NTR) of primary and not received radiation therapy (RT). Patient had lung metastasis and started on hydroxy urea. Patient had a stable disease after a follow-up of 3 months. Solitary fibrous tumor patient underwent subtotal resection (STR) and received RT. Patient had lung metastasis and defaulted systemic therapy. Patient had stable disease after a follow-up of 1.5 years. Medulloblastoma patient underwent NTR and RT. Patient had bone metastasis and was lost to follow-up.

Two had metachronous metastases: One with Glioblastoma and one with solitary fibrous tumor. Both had surgery and RT to primary site. Glioblastoma patient had bone metastasis and received palliative RT and died after a follow-up of 8 months from diagnosis. Solitary fibrous tumor patient developed lung metastasis and local recurrence. Patient underwent surgery for both, reirradiation for local recurrence and started on Pazopanib. Has follow-up of 7 years from diagnosis and presently had progression of lung metastases.

Conclusion: In our series the most common site of metastasis was lungs. Extraneural metastasis has a dismal outcome. Clinicians should keep in mind that the primary CNS tumors can metastasize in order to diagnose extraneural metastases early.

Ependymosarcoma harboring C11orf95�RELA fusion transcript: Report of two cases and review of literature

Swati Mahajan, Aruna Nambirajan, Agrima Sharma, Vaishali Suri, Hardik Sardana, Manoj Phalak, Ajay Garg, Mehar Chand Sharma; All India Institute of Medical Sciences (AIIMS), New Delhi, India

Introduction: Ependymomas are relatively rare glial tumors of the central nervous system that supposedly arise from the cells lining the ventricles and central canal within the spinal cord. Ependymosarcoma (ES) is a newly introduced tumor entity of uncertain prognosis characterized by a rare phenomenon of malignant mesenchymal transformation arising within an ependymoma. These tumors were predominantly supratentorial, extra ventricular and occur across a wide age range (2–63 years). Histomorphology shows an admixture of glial and mesenchymal component, most commonly being fibrosarcoma, malignant fibrous histiocytoma, osteosarcoma, liposarcoma, or rhabdomyosarcoma in decreasing order of frequency. The glial component is usually of astrocytic differentiation, although in extremely rare instances, oligodendroglial, or ependymal differentiation has also been noted. They are surgically challenging tumors with a high incidence of morbidity and mortality. Study of literature shows only 22 cases of ES reported till date.

Objectives: Here we are reporting two cases of primary ES harboring novel C11orf95 –RELA fusion transcript.

Materials and Methods: A 25-year-old female and a 17- year- old male, both presented with progressive and sequential neurological deficits over a period of 5-8 months. Histopathological examination revealed a biphasic tumor comprising of anaplastic glial component intermingled with the sarcomatous component. The glial component displayed cords, nests and wavy fascicles of elongated hyperchromatic cells that were occasionally interrupted by perivascular pseudorosettes and papillae. Non-glial sarcomatous component displayed interlacing fascicles of pleomorphic oval to spindled cells with vesicular nuclei and moderate amount of eosinophilic cytoplasm. Brisk mitotic activity and extensive areas of necrosis were present throughout the tumor. Glial component showed diffuse positivity for GFAP, PanCK and dot-like positivity for EMA and EBP-50. In contrast, sarcomatous component failed to stain for GFAP and PanCK, however showed diffuse positivity for vimentin and focal positivity for EMA and EBP-50. IHC staining for L1CAM, a downstream marker for the C11orf95–RELA fusion showed strong membranous immunopositivity in both the components, which was further confirmed by sanger sequencing. Based on characteristic histopathological, immunohistochemical and molecular findings a final diagnosis of ES was rendered.

Conclusion: Ependymosarcomas are rare and enigmatic tumors with an evolving understanding of their molecular profile and its significance. Sarcomatous change in ependymoma is rare and likely to be misdiagnosed. There is a need to explore the molecular alterations underlying the sarcomatous transformation through genome-wide molecular profiling to determine optimal patient management and placement within appropriate clinical trials.

Extended adjuvant temozolomide (>6-cycles) compared to standard 6-cycles of adjuvant temozolomide in newly diagnosed glioblastoma: Systematic review and meta-analysis of randomized controlled trials

Riddhijyoti Talukdar, Abhishek Chatterjee, Sadhana Kannan, Vijay Patil, Tejpal Gupta; Tata Memorial Center, Mumbai, Maharashtra, India

Introduction: The contemporary standard of care for patients with newly-diagnosed glioblastoma is maximal safe resection followed by focal conformal radiotherapy with concurrent temozolomide (TMZ) and 6-cycles of adjuvant TMZ chemotherapy (Stupp regimen). Despite limited evidence for the benefit of extended adjuvant TMZ (beyond the standard 6-cycles), it continues to be used variably in different healthcare settings across the world. The aim of this systematic review and meta-analysis was to compare survival outcomes of extended adjuvant TMZ (>6-cycles) versus standard 6-cycles of adjuvant TMZ in patients with newly-diagnosed glioblastoma.

Materials and Methods: Only prospective clinical trials randomly assigning adult patients with newly-diagnosed glioblastoma to standard 6-cycles of adjuvant TMZ or extended adjuvant TMZ (>6-cycles) were eligible. Individual patient data were extracted from the published survival curves using WebPlot digitizer. Hazard ratio (HR) for overall survival (OS) with corresponding 95% confidence interval (CI) was estimated from the reconstructed survival curves and reconciled with the reported outcomes. Random-effects model was used for pooling HRs from individual randomized controlled trials (RCTs) to compute summary estimates in the meta-analysis.

Results: Systematic review of the literature identified 4 RCTs comparing standard 6-cycles of adjuvant TMZ versus extended adjuvant TMZ (>6-cycles) with extractable data from 258 glioblastoma patients reported in 3 studies. Extended adjuvant TMZ was associated with a 23% relative reduction in the risk of death (pooled HR=0.77, 95%CI:0.40-1.46; P =0.42) compared to standard adjuvant TMZ (Figure 1) which was not statistically significant.

Conclusions: Extended adjuvant TMZ (>6-cycles) does not provide any survival benefit over the standard 6-cycles of adjuvant TMZ in patients with newly-diagnosed glioblastoma.

Lymphoplasmacyte-rich meningioma: A rare pathological entity

Jyoti Pandey, Surabhi Tyagi; Mahatma Gandhi Medical College

Introduction: Lymphoplasmacyte-rich meningioma is a rare variant of meningioma, which is characterized by extensive inflammatory cell infiltrate of lymphocytes and plasma cells often obscuring the Meningothelial component.

Objective: The objective of our study was to study the clinicopathological characteristics and differential diagnosis of LPM.

Materials and Methods: We report a case of a 25-year-old male who presented with a space-occupying lesion in the right fronto-parietal convexity. The surgical specimen comprised of a grey-brown mass measuring 3 cm x 3 cm x 1.2 cm. At microscopy spindle-shaped tumor cells in whirlpool arrangement associated with infiltration by lymphocytes, plasma cells and macrophages and inconspicuous meningothelial component were identified.

Positive immunoexpression for EMA, Vimentin, CD45, CD3 and negative immunoexpression for GFAP.MIB-1 was 3-5% in the highest proliferating areas.

Histopathological and immunohistochemical examination finally helped in reaching the diagnosis of Lymphoplamacytic-rich meningioma with brain invasion and marginally MIB-1 LI.

Results: Following the histological and IHC assessment; diagnosis of lymphoplasmacyte rich meningioma with brain invasion and marginally MIB-1 LI.

Conclusion: Lymphoplasmacytic-rich meningioma is a very rare variant of intracranial meningiomas, featured by massive inflammatory cell infiltration and often, a less proportion of meningothelial tumorous elements. Surgical resection is still the primary treatment.

Histopathological and IHC helped in the proper characterization, staging and grading of the tumor and in differentiating LPM from other intracranial lesions for further management of the case.

Keywords: Histopathology, immunohistochemical, lymphocyte, meningioma, plasma cells

Carcinoid tumor of the spinal cord: A rare pathological entity

Mudita Sharma, Surabhi Tyagi; Mahatma Gandhi Medical College and Teaching Hospital

Introduction: Carcinoid tumors are rare in CNS. Carcinoid tumors (CTs) are neuroendocrine tumors derived from the enterochromaffin cells, mainly in bronchi and gastrointestinal tract. CNS involvement is rare. It occurs as metastasis either to the brain or spinal cord. In the spinal cord, the primary tumors involve the sacrum and coccyx.

Objectives: To study a rare case of Carcinoid tumor of spine involving the C7-T2 vertebrae.

Materials and Methods: A 55-year-old male presented with complaints of difficulty in walking and weakness in bilateral lower limb (R >L). The surgical specimen comprised of several gray white to gray-brown soft tissue pieces collectively measuring 5x3x1 cm. On H and E diagnosis of neuroendocrine tumor possibly atypical carcinoid was made and IHC suggested.

On IHC there was Positive immunoexpression for neurofilament, chromogranin, and negative immunoexpression of synaptophysin and cytokeratin. MIB1 was 18%–20% in highest proliferating areas.

Result: Diagnosis of Neuroendocrine tumor, Atypical Carcinoid was made and confirmed on IHC.

Conclusion: Primary spinal carcinoid tumor is rare. Definitive diagnosis from other common brain tumors is only possible after histopathology and immunohistochemistry. Radiotherapy and chemotherapy are advised as per the tumor grade and stage.

Keywords: Carcinoidtumor, histopathology, immunohistochemical, neuroendocrine

Clinical, etiological, and radiological profile of posterior reversible encephalopathy syndrome (PRES) from a tertiary care center

Asim Shrestha, Suhani Shah, Anaita Hegde, Aoyon Sen, Soonu Udani, Sujata Mushrif, Ruchira Mishra, Purna Kurkure, Hiren Panwala, Divyata Hingwala; Department of Neurology, SRCC Children Hospital, Department of ICU, SRCC Children Hospital

Background: PRES is characterized by seizures, altered sensorium with typical transient parieto-occipital lesions on MRI.

Materials and Methods: This was a retrospective study of patients admitted with PRES in the ICU from Jan 2020 to Feb 2021. Etiological, clinical and radiological features with relation to outcome were studied.

Results: 14 cases were admitted with features of PRES. Mean age was 9.25 years(2- 17 years). The clinical presentations were seizure (12/14), altered sensorium (2/14) and isolated nystagmus (1/14). Hypertension was noted in 10/14.

Commonest etiology was hemato-oncological(10/14), followed by renal (3/14) and gastroenterological(1/14). The hemato-oncological cases were acute lymphoblastic leukemia(3/14), thalassemia(3/14), hemophagocytic lymphohistiocytosis (2/14) and aplastic anemia(2/14). Of these, 3 had already undergone hematopoietic stem cell transplant- thalassemia(2) and aplastic anemia(1). Drugs that could be contributory were: steroids(11/14), cyclosporine(8/14), mycophenolate mofetil (2/14), cyclophosphamide(1/14) and tacrolimus(1/14).

On neuroimaging, classical subcortical lesion was noted in 12/14 and cortical in 12/14, with 10/14 showing both. Distribution was parieto-occipital (14/14), frontal (7/14) and temporal (7/14). Cerebellar and basal-ganglia lesions were seen in 3/14 and 2/14, respectively. Microbleed and macrobleed were noted in 6 and 1 case. 50% with intracranial bleed had poor outcome.

3/14 died of primary pathology complications. 1/14 had severe morbidity secondary to drug-induced PRES. 10/14 had complete recovery.

Conclusion: PRES is a neurological entity commonly seen with hemato-oncological condition. Etiology is commonly drug-induced. Hypertension is not always associated. Neuroimaging predominantly shows subcortical, cortical involvement. Parieto-occipital lobe is usually involved along with frontal and temporal lobes. Bleeds are associated with poor outcome. PRES is not as benign as previously thought.

Sacrococcygeal chordoma

Chandramouli Balasubramanian, Amit Kumar Gosh, Aditya Mantry, MM Gosh; Institute of Neuroscience Kolkata, Kolkata, West Bengal, India

We present a very rare case of sacrococcygeal chordoma which was operated in collaboration with plastic surgeon, when a 5 kg tumor was removed from around the sacrum and coccyx.

The patient has already undergone 3 previous operations for recurrent tumor in the same region. She has also received radiotherapy before this operation. This fourth operation was particularly challenging as the tumor was extending around the gluteus Maximus and into the pararectal space. Near-total removal of the tumor was performed with preserved sciatic nerve and anal sphincter function. We review the current treatment methods for the same.

Chordoma is a rare, low-grade malignant bone tumor arising from primitive notochord remnants of the axial skeleton. As the spine develops, notochordal remnants are concentrated at nucleus pulposus. This explains why chordomas are in the midline or the paramedian location.Their incidence rate is 0.1/100,000/year. Chordomasinvolve the sacrococcygeal region in 50%–60% of the cases and the clivus or the spheno occipital region in 30%–35% cases. They account for over 40% of all sacral tumors. Local invasiveness and destructiveness are characteristic features of the disease.

Complete surgical excision is the main therapeutic modality able to affect a cure. Imaging techniques in particular MRI play a crucial role in surgical planning. Prognosis depends on the resection of the tumor in surgery and postoperative treatment. Although metastases also may occur, most patients who succumb to the disease do so because of local recurrences. Five-year survival rate is 51% and 10-year survival rate is 35%. The curative treatment of chordomas is en bloc surgical resection with negative margins. It is associated with long-term disease control and potential cure. But most often, due to the location, size and the extent of the tumor, margin-free resection is not possible. The expected local failure rate in case of a marginal resection is 70%. Chemotherapy is debated. Few studies show that some patients responded to 800 mg Imatinib daily.

Clinical and histopathological spectrum of cranial small round cell tumors: An experience from a tertiary care center

Ravi Jacob, Pooja Gupta, K.B. Shankar, Fouzia Siraj; ICMR-National Institute of Pathology

Introduction: Small round cell tumors (SRCT) are a group of malignant neoplasms with minimal or no differentiation characterized by presence of round cells with high nuclear-cytoplasmic ratio. Although SRCT can occur in any part of the body, involvement of central nervous system (CNS) is uncommon.

Aim: We aimed to study the clinicopathological spectrum of cranial SRCT diagnosed in our institute over a period of four years (2016–2019).

Materials and Methods: A retrospective review of medical records (2016-2019) with morphological diagnosis of SRCT in cranium was made. Both intra-axial and extra-axial tumors were included. A total of 60 cases were retrieved and the clinical and histopathological features were studied. Special cytochemical staining and immunohistochemistry were performed, where needed.

Results: The mean age of presentation was 18.4 years (range, 1-60 years), with a male to female ratio of 2.5:1. The most common site was posterior fossa of brain (n = 28, 47%) followed by dorso-lumbar spine (n = 9, 15%). The most common tumor was medulloblastoma (n = 29, 48.3%) followed by Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumor (pPNET) (n = 11, 18.3%), Non-Hodgkin lymphoma (NHL) (n = 9, 15%), neuroblastoma (n = 3, 5%) and CNS embryonal tumor NOS (n = 2, 3.3%). One case each of atypical tetroid rhabdoid tumor (ATRT), rhabdomyosarcoma, pineoblastoma, melanoma, rhabdomyosarcoma and undifferentiated pleomorphic sarcoma was also documented.

Conclusions: SRCTs have a variable age of presentation. Their incidence in cranium is uncommon as compared to other systems of the body. The histopathology of these lesions on light microscopy is overlapping, posing a great diagnostic dilemma for the pathologist. The use of ancillary techniques like immunohistochemistry helps in arriving at the correct diagnosis. Treatment strategy and tumor prognosis also vary along the entire spectrum of SRCT, thus making exact characterization essential for proper management.

Key Message Small round cell tumors have a variable clinical presentation. It is important to use histopathology with the aid of ancillary techniques for their correct diagnosis and management. This study highlights the clinical and histopathological spectrum of cranial based small round cell tumors diagnosed in our institute over a period of four years.

Giant cell glioblastoma: A case series of clinical and pathological profile of an uncommon glioma

Megha Panchal, K. B. Shankar1, Fouzia Siraj; Department of Pathology, ICMR-National Institute of Pathology, New Delhi, India, 1Department of Neurosurgery, VMMC and Safdarjung Hospital, New Delhi, India

Introduction: Giant cell glioblastoma (GC-GBM) is an uncommon subtype of glioblastoma (GBM). As a result, the epidemiology, natural history, and factors associated with outcome are not well defined.

Aim: We aimed to study the clinicopathological spectrum of giant cell glioblastoma in our institute over a period of five years.

Materials and Methods: A retrospective review of medical records (2014-2019) with morphological diagnosis of GC-GBM was done. The clinical and histopathological features were studied and immunohistochemistry was performed, wherever needed.

Results: A total of 154 cases of glioblastoma were diagnosed out of which 8 were found to be giant cell glioblastoma (5.1%). In our series the average age of presentation was 25 years with male preponderance (male:female ratio = 3:1). Most frequent location was parietal lobe followed by frontal lobe. All patients presented with complaints of headache and vomiting. Postoperative mortality rate was high in our series with 90% patients dying within 6 months to 1year after surgery. However, this was attributed to complications secondary to surgical procedure. Morphologically tumors showed predominantly bizarre giant cells in addition to all other features of GBM like nuclear atypia, endothelial proliferation, necrosis and increased mitosis. Immunoreactivity for GFAP, TP53 and variable ki67 proliferative index (3%–10%) was noted. Contrary to published literature, strong IDH1 positivity was observed in 40% of our cases.

Conclusion: Giant cell glioblastoma is an uncommon tumor associated with younger age and better survival compared to classical glioblastoma. However, given the rarity of this tumor, the pathobiology and best management practices are yet to be ascertained.

Inflammatory indices in diffuse gliomas (InDiGo): A prospective observational study

Yamini Baviskar, Abhishek Chatterjee, Prakash Shetty, Aliasgar Moiyadi, Ayushi Sahay, Sridhar Epari, Archya Dasgupta, Goda Jayant Sastri, Tejpal Gupta; Tata Memorial Hospital, Mumbai, Maharashtra, India

Objectives: To assess differences in baseline (pre-surgical) systemic inflammatory markers between patients with glioblastoma (GBM) compared to diffuse lower grade glioma (LrGG).

Materials and Methods: Forty-five adults with clinicoradiological suspicion of diffuse glioma and no history of thrombo-embolism, immune-deficiency, recent infection or corticosteroid intake underwent venous sampling after written informed consent for complete blood counts, serum biochemistry including albumin, C-reactive protein (CRP), ferritin, fibrinogen, and erythrocyte sedimentation rate (ESR) to calculate various systemic inflammatory ratios and indices. Subsequently, all patients underwent neuro-surgical debulking and were treated with standard adjuvant therapy as appropriate. Mean values of baseline inflammatory indices were compared between LrGG (WHO grades II-III) and GBM (WHO grade IV) using unpaired t test and Mann–Whitney U test for normal and non-normal distribution of data, respectively. A value of P < 0.05 was considered statistically significant Receiver operating characteristics (ROC) curves were generated for indices that were statistically significant to define optimal cut-offs.

Results: Among the various inflammatory indices studied, neutrophil-to-lymphocyte ratio (NLR, P =0.002), monocyte-to-lymphocyte ratio (MLR, P = 0.007), granulocyte-to-agranulocyte ratio (GrAgr, P = 0.003), systemic inflammatory index (SII, P = 0.005) and prognostic nutrition index (PNI, P = 0.04) were significantly different between LrGG and GBM. Optimal cut-offs obtained from the ROC curves to differentiate GBM from LrGG were 2.83, 0.22, 1.94, 758.9, and 52.75 for NLR, MLR, GrAgr, SII, and PNI, respectively.

Conclusion: Systemic inflammatory markers are significantly higher and immuno-nutritional index significantly lower at baseline (prior to surgery) in patients with GBM compared to LrGG.

Telomerase reverse transcriptase promoter mutations in a cohort of meningiomas

Julie Joseph, Geeta Chacko, Rekha Pai, Ari G Chacko, Bashir Ahmed, Abarna R, Mahasampath Gowri; CMC, Vellore, Tamil Nadu, India

Objectives: To determine the prevalence of TERT promoter mutations assessed by droplet digital PCR (ddPCR) in meningiomas, its association with WHO grading, progression free survival (PFS) and overall survival (OS).

Materials and Methods: Fifty five cases of meningiomas, with a minimum 5 year follow-up, were included in this cross sectional observational study. TERT promoter mutation analysis was performed using the ddPCR technique. The association of TERT promoter mutation with histological grading, overall and progression-free survival was analyzed.

Results: There were 22 (40%) males and 33 (60%) females. Age of the patients ranged from 10 to 68 years with a mean age of 40.33 years. The most common location of meningioma was the cerebral convexity. A total of 46 patients (83.6%) underwent complete resection. There were 28 WHO grade I meningiomas, 18 WHO grade II and 9 WHO grade III meningiomas. Three meningiomas were recurrent of which one was WHO grade III and two were WHO grade II with no histological progression from the previous biopsy. None of the cases showed TERT promoter mutation by ddPCR.

Conclusion: TERT mutations are uncommon in de novo meningiomas, even in those of a higher histological grade. Larger studies are required to explore the possibility of TERT promoter mutations being population specific as well as the contribution of heterogeneity to the detection of mutations.

Seizure outcome in dysembryoplastic neuroepithelial tumors: A series of 15 cases and review of literature

Soumen Kanjilal, Anant Mehrotra, Suyash Singh; Sanjay Gandhi Post Graduate Institute of Medical Sciences

Objective: To analyze seizure-outcome of surgically managed DNET patients and highlight the factors that predicts seizure-control and long-term consequence after surgery.

Materials and Methods: A retrospective analysis of the prospectively maintained surgical database of DNET was done and operated patients from January 2015 and February 2020 were identified. Patients presenting with medically refractory seizures (ILAE 2017) were included in our study. Gross-total excision of the tumor had always been the intention in each case. The seizure outcome was graded according to Engel classification.

Results: Among 15 patients, the mean duration of seizure was 49.9 + 38.6 months. 12 patients underwent gross total excision. The histopathological slides in eleven patients had simple DNETs, two had complex DNETs, and two patients had composite/ mixed DNETs, respectively. At the mean duration of follow-up of 52-months (range 2 to 109), 12 patients (80%) achieved Engel Class I, 2 (13.3%) patient achieved Engel Class II and 1 patient (6.7%) achieved Engel Class III outcome. There were 10 patients (66.7%) with complete seizure freedom (Engel Class IA) post-operatively. Patients operated at younger age seem to have poor seizure outcome but these children had a comparatively longer pre-operative seizure duration (P =0.23).

Conclusion: DNET forms majority of low-grade epilepsy associated neuro-epithelial tumors. Complete excision of DNETs, shorter duration of seizure and age of presentation are important factors for seizure control. Although lesionectomy alone may suffice in most of the cases, a more aggressive surgical strategy such as extended resection may appear rationale for medically refractory intractable epilepsy.

An unusual presentation of a common disease

Jyothis P Jose, E Senthilkumar1, SGD Gangadharan1; Department of Medical Oncology, Madras Medical College, 1Madras Medical College and Research Institute

Paraneoplastic cerebellar degeneration (PCD) is an uncommon heterogeneous group of disorders that are indicators of the underlying occult malignancy. We report a case of 62-year-old female that presented with subacute cerebellar dysfunction, before any evidence of tumor, but early diagnostic workup and vigilant clinical examination helped us to find the underlying high-grade ovarian adenocarcinoma. The prompt treatment and aggressive rehabilitation resulted in partial recovery of the neurologic symptoms.

Middle cranial fossa epidermoid cyst: A case report

Neha Saxena, Sudha Iyengar, K.S. Mangal, Nishant Gupta; Gajra Raja Medical College

Objectives: • To study the histopathological examination of the submitted tissue from operated case of left middle cranial fossa space-occupying lesion.

• To correlate histopathological findings with MRI and clinical features.

Materials and Methods: Patient is traced from neurosurgery ward and history is taken. According to that, a 29-year-old female presented to the OPD with complaints of vertigo and headache since 1 year along with hearing loss and blurring of vision since 1 month. Magnetic Resonance Imaging (MRI) revealed well-defined lobulated extraxialT1 heterogeneously hypointense and T2 heterogeneously hyperintense mass in left middle cranial fossa. Patient is operated in J.A.H group of hospital. Histopathological examination was done after surgery and diagnosed as epidermoid cyst.

Result: Histopathological examination of the tissue submitted on microscopic examination show a cyst which is lined by stratified squamous epithelium, underneath seen keratin flakes. An epidermoid tumor arises from inclusion of ectodermal epithelial elements.

Conclusion: Epidermoid cyst is a rare congenital tumor of the central nervous system. Epidermoid cysts are benign slow growing more often extra-axial tumors that insinuate between brain structures. Embryo logically, they are derived from ectodermal inclusions during neural tube closure from the third to the fifth weeks of embryogenesis. They frequently occur at the Cerebellopontine angles and parasellar regions, insinuating between brain structures. Conversely, epidermoid cysts in intraparenchymal or intradiploic locations are very rare, accounting for less than 5% of all intracranial epidermoid.

Awake craniotomy in young adult for right motor strip glioma

Amol Kaswa, Pooja Kaswa; Asian Noble Hospital, Ahmednagar, Maharashtra, India

Objective: An awake craniotomy facilitates radical excision of eloquent area gliomas with decreased iatrogenic postoperative neurological deficits. In this article, we review recent evidence for utility of awake craniotomy in resection of right-sided motor strip glioma in a young adult.

Materials and Methods: In our study, the patient was a 27-year-old farmer, who presented with headache and complex partial seizures. Preoperative imaging included MRI brain with and without gadolinium contrast, diffusion tensor imaging and functional MRI.

MRI Brain showed a lesion with mild-to-moderate contrast enhancement in right frontal lobe involving the premotor region. The lesion measured approximately 5 cm × 3.8 cm × 4.8 cm in AP, transverse and craniocaudal dimensions, respectively. Tractography evaluation showed posterior displacement of right corticospinal tract by lesion with an intervening distance of 1–2 mm. Function MRI showed hand motor area located posterior to posterior margin of lesion with intervening distance of 5 mm.

Patient was posted for awake craniotomy with electrophysiological monitoring with proper preoperative counseling. Our anesthesia technique involved bilateral scalp block with bupivacaine 0.25% and occasionally fentanyl boluses. The head was positioned on horse shoe and we did not use invasive monitors such as urinary catheter or arterial lines. 5mins prior to cortical mapping the fentanyl infusions were stopped. Right frontal craniotomy was done. The motor area was mapped using a probe with two tips separated by 6–10 mm and 100 µs square wave pulse. The eletromyographic recording was noted and patient was informed to report any altered sensation he experienced in his left-hand function. Diminution of left-hand function suggested that the brain area probed was eloquent area. Using this technique radical excision of tumor was carried out. Surgery lasted for 3 h. Postoperatively patient had transient mild weakness in left-hand grip which gradually improved within 12 h. Patient was discharged 3days after surgery with no neurological deficits. His histopathological report was astrocytoma – WHO Grade II.

Results: Patient tolerated awake craniotomy very well. Radical excision of tumor was done with transient left-hand weakness which completely recovered.

Conclusion: Awake craniotomy for excision of eloquent area glioma enable accurate mapping of motor area as well as continuous neurological monitoring during tumor removal. Furthermore, positive responses on white matter stimulation indicate close proximity of eloquent cortex and projection fibers. This should alert the surgeon to possibility of postoperative deficits to change the surgical strategy. Thus the surgeon can maximize tumor resection and minimize neurological deficits.

Askin tumor: A case report and treatment strategies

Dawn Antony, Chong Chee Fui, Norehan Johari; PJSC

Background: Ewing’s Sarcoma of the thoraco-pulmonary region are described as Askin tumor, after being first reported by Askin et al. in 1979. Being rare tumors, diagnosis and treatment remain a challenge due to the absence of validated standard treatment guidelines.

Case Report: We present a 9-year-old male child who presented with sudden onset of lower limb weakness. He has a history of inhalation therapy for asthma. Scans showed a large tumor in the right hemithorax extending through the right neural foramen at T7/8 to the epidural space causing severe cord compression from T6 to T8 level.

He underwent laminectomy and total excision of the spinal component of the tumor. He was then given chemotherapy to downsize the thoracic component. This was later excised thoracoscopically. He was then continued on chemotherapy. On last follow-up he has not made any significant neurological recovery but remains disease free as evidenced by scans.

Conclusion: Askin tumors should also be included in the differential diagnosis of dumbbell tumors as they can mimic nerve sheath tumor both clinically and radiologically. Treatment strategies should be directed to reduce local recurrence and distant metastasis.

Machine learning tools for complication prediction in spine surgery: A review

Urja Parekh, Hrishikesh Sarkar; NMIMS Sunandan Divatia School of Science

Background: Intra- and postoperative complications occur in about 50% of the patients undergoing spine surgery. This bears a burden to the patient’s quality of life and also increasing the cost of healthcare. Despite vast clinical experience, the risk estimate for complications provided by surgeons is rather inaccurate. This is because of the number and variability of features involved in the process, combined with the psychological factors associated with spine disease pathologies.

Objective: The aim of this review was to evaluate whether machine learning techniques can predict complications in spine surgery with high accuracy, sensitivity and specificity.

Materials and Methods: Literature for the same was gathered from Google Scholar from 2016 to 2020, using search terms “Complication Prediction” AND “Spine Surgery” AND “Machine Learning”. Area under the receiving operating characteristic curve (AUC) was the primary outcome of our study.

Results: Nineteen of 55 papers were included that focused on complication prediction in spine surgery. A total of 4,302,693 patients (mean age 57.30 years and 56.91% females) were analyzed. The studies focused on complication prediction in general spine surgery and/or specific surgical techniques or spinal diseases. The risk estimate for outcomes such as back pain, leg pain, functional status, cardiac dysfunction, congestive heart failure, pulmonary complication, pneumonia, neurologic complications, urinary tract infections, invasiveness of surgery and mortality were evaluated. Commonly employed machine learning algorithms were random forest, logistic regression and gradient boosting. A feature set as large as 300 variables was used predictive modeling. The best model for overall complication prediction was logistic regression. Maximum AUC obtained was 0.99 for adverse event prediction in general spine surgery via the Naïve Bayes algorithm. Highest sensitivity (100%) was obtained via logistic regression model for prediction of ischemic optic neuropathy in patients undergoing spine surgery. Highest specificity (99%) was obtained for overall complication prediction via Logistic regression, Adaboost, Stochastic Gradient Descent. However, these studies were conducted retrospectively and thus, made the nature of the findings associational rather than causational, reducing generalizability of the results for the larger population.

Conclusion: Machine learning tools are effective for prediction of intra- and post-operative spine surgery complications since they take into consideration large number of variables for risk estimation. However, improvements in models are required to increase accuracy, sensitivity and specificity and reduce overfitting. Currently, these tools can only be used as an adjunct for decision-making to reduce complications in surgery, and complement the judgement of surgeons, not replace it.

Lessons learned from spinal dermoid and epidermoid cyst with clinical insights into neural tube closure models

Yashveer Singh, Ved Prakash Maurya; Sanjay Gandhi Post Graduate Institute of Medical Science

Objectives: The spinal dermoid and epidermoid cysts (SDEC) are rare entity comprising less than 1% of pediatric intraspinal tumors. This study aims to extrapolate the clinico-radiological data in order to identify the most plausible neural tube closure model in human and provide a retrospective representation from our clinical experience of over two decades from a tertiary care center.

Materials and Methods: We collected the details of all histologically proven, newly diagnosed primary SDEC who underwent excision over the last 20 years. Secondary or recurrent lesions and other spinal cord tumors were excluded. Surgical and follow-up details of these patients as well as those with associated spinal dysraphism were reviewed. Clinical and radiological follow-up revealed the recurrence in these inborn spinal cord disorders.

Results: Total 73 patients were included retrospectively having a mean age of 22.4–13.3 years and 41 (56.2%) cases fall in first two decades of life. 24(32.9%) dermoids and 49(67.1%) epidermoid comprised the study population and 20 of them had associated spinal dysraphism.

We noticed about 8 folds higher incidence of epidermoids (n = 23) in females than dermoids (n = 3). In this study, 44(60.3%) patients presented in their 2nd and 3rd decades while 13(17.8%) patients were diagnosed during the first decade of life. The distribution of SDEC was commonest in lumbosacral region (n = 30) which was ten times more common than in the sacral region (n = 3). Bladder dysfunction 50(68.5%) and pain 48(65.7%) were the commonest presenting complaints.

Bladder involvement was more frequent in epidermoids 31/50(62%) as compared to the dermoids 19/50(38%) (P = 0.19). During follow-up visits, 40/48(83.3%) cases showed sensory improvement while 11/16(68.7%) regained normal bowel function. There was no surgical mortality with eight recurrence till the last follow-up.

Conclusions: The protracted clinical course of the spinal inclusion cysts mandates a long term follow-up. The results of our study support the multisite closure model and attempts to provide a retrospective reflection of neural tube closure model in humans by using SDEC as the surrogate marker of neural tube closure defect.

Clinicoradiological spectrum of CNS paragangliomas: A series of an uncommon masquerade of common entities

Parul Jain, Sumit Thakar, Nandita Ghoshal; SRI Sathya Sai Institute of Higher Medical Sciences

Objectives: Paraganglioma that involves the CNS may mimic clinicoradiologically many other commoner entities. This study presents a wide view of the clinical, radiological, histomorphological spectrum and rare associations that can occur concurrently with this lesion.

Materials and Methods: This series includes cases where the brain and spinal cord paragangliomas were diagnosed and managed in Sri Sathya Sai Institute of Higher Medical Sciences, Bangalore over 15 years (2005–2020). The clinical details, radiology, and pathology findings were retrieved and reviewed.

Results: Of the 13 cases, the mean patient age was 35 years (13-57) including 10 males and 3 females. The most common site of the infliction was the spine (9/13). Clinicoradiologically 6 were diagnosed as neurofibroma/schwannoma, one as myxopapillary ependymoma and the other two were recurrences. On MRI, paragangliomas are T1 iso to hypointense and hyperintense on T2 with contrast enhancement and characteristic peripheral vascularity. One case had associated superficial craniospinal hemosiderosis.

Two cases involved the sellar region where they were misdiagnosed as non-functioning pituitary adenomas. On imaging, in 1 case arteriovenous malformation was also kept as a differential, owing to the large perilesional tortuous arterial flow voids.

One case each was noted in the forebrain and at the CP angle. Clinicoradiologically the intra-axial lesion mimicked metastasis/meningioma/vascular malformation/hemangiopericytoma/glioma. In the same case, there was a history of excision of carotid body tumor 8 years back. The lesion at CP angle was diagnosed as glomus body tumor and it also had an association with Dandy–Walker malformation.

The final diagnosis was conferred on histopathology relying on the idiosyncratic histomorphology consisting of large cells arranged in zellballen pattern, characteristic reticulin network, and immunopositivity for chromogranin and synaptophysin.

Disease outcome depends on the extent of tumor excision, as recurrences are common in a partially excised tumor. The extent of surgery is dependent upon the site, local factors, and the distinctive peripheral vasculature.

Conclusions: Though benign in nature, diagnosis and management of paraganglioma in the CNS pose difficulty at each step. It is important to keep it as a differential with other commoner entities. Rich vascularity of the tumor is a soft signature radiologically. Histopathology is characteristic but sometimes varied morphological features may pose diagnostic difficulty and aid of immunohistochemistry may be sought for. Also one needs to keep in mind that various syndromic associations of this benevolent lesion might warrant further genetic analysis.

Integrated classification of pediatric medulloblastomas (histologically and genetically defined) with prognostication, using immunohistochemical markers

Badrinath T, Chacko AG, Moorthy RK, Joseph BV, John RR, Rajesh B, Bindra MS, Chacko G; Christian Medical College, Vellore, Tamil Nadu, India

Objectives: To classify pediatric medulloblastomas based on histological and genetically defined WHO CNS, 2016 criteria using immunohistochemical markers, β-catenin, GAB-1, YAP-1 and p53 and ascertain the prevalence of each subtype.

Materials and Methods: A cohort of 111 cases of pediatric medulloblastoma (0–18 years) from January 2009 to June 2019 were included in this study. A detailed review of the hematoxylin and eosin-stained slides and histological and molecular subgrouping was done with the help of four immunohistochemical markers, β-catenin, GAB-1, YAP-1 and P53. Data analysis was done using SPSS software and association between the categorical variables was done using a chi-square statistics/ Fisher exact test (P < 0.05, significant).

Results: Pediatric medulloblastomas were seen predominantly in the midline with a median age of 8 years (M:F=2.5:1). On histological subgrouping, 70.3% were classic, 13.5% each were of the desmoplastic/nodular and large cell/anaplastic type and 2.7% were medulloblastomas with extensive nodularity. On molecular subgrouping non-WNT/non-SHH formed the largest subgroup with 27% cases, followed by 23.42% of the SHH pathway and, 12.61% were subtyped as belonging to the WNT pathway. There were 36.93% cases that were discordant and could not be classified into any of the genetically defined subgroups by immunohistochemistry. We developed a cost-effective algorithm, where we proposed that GAB-1 and YAP-1 immunohistochemistry be done as the first step. If both these markers are positive, the tumor can be classified as SHH subgroup and if both are negative, it can be labeled non-WNT/non-SHH subgroup,. Further Tp53 immunohistochemistry can be done to classify the SHH-activated as Tp53 mutated or wild type. In cases with isolated YAP-1 positivity, beta-catenin immunohistochemistry can be done as the second step, if positive, these tumors can be classified as WNT activated medulloblastomas. If beta-catenin is negative, the tumor becomes discordant to classify by immunohistochemistry alone and requires further molecular analysis. Additionally, tumors with isolated GAB-1 positivity are also considered discordant by immunohistochemistry and require further molecular analysis.

Conclusion: The panel of immunohistochemical markers β-catenin, GAB1 and YAP1 markers are useful in molecular subgrouping of medulloblastomas and utilizing them in an algorithmic fashion can prove to be economical.

Factors predicting “postoperative cerebellar mutism syndrome” after posterior fossa tumor excision in pediatric patients

Aanchal Datta, Ashutosh Kumar, Suyash Singh, Kamlesh Bhaisora, Arun Kumar Srivastava, Jayesh Sardhara, Kuntal Kanti Das, Anant Mehrotra, Awadhesh Kumar Jaiswal, Sanjay Behari; SGPGI

Background: Central tumor location, brainstem involvement, and medulloblastoma have been implicated as risk factors for pediatric postoperative cerebellar mutism syndrome (pCMS) by most researchers. However, conflicting results have been reported for surgical factors like the extent of excision, surgical approach, and need for CSF diversion. The role of emergency or elective surgery is also not well established.

Aim: To assess the role of surgical factors as a risk for pCMS. The secondary variables assessed include patient profile, histopathology, and radiological features. We also evaluated the effect of pCMS patient survival.

Materials and Methods: A total of 162 consecutively operated patients of posterior fossa tumor from July 2012 to March 2020 with age less than 16 years were included in the study. The study population was divided into two cohorts: the pCMS group and the non- pCMS group for statistical analysis of associated factors. Chi-square test was used to compare the mean distribution of study parameters among the groups. A value of P < 0.05 was considered statistically significant.

Results: In the sample size of 162 patients, N = 129 patients were included for analysis. There were 11 patients in the pCMS group. Emergency surgery was found to be a significant risk for pCMS (P = 0.021). There was a trend towards statistical significance for the need for preoperative CSF diversion. The extent of resection and surgical approach was not found to be statistically significant factors. Central tumor location and contrast enhancement were significant radiological risk factors (P < 0.05).

Conclusion: Aggressive tumor resection causing iatrogenic injury to cerebellar circuitry and brainstem is the most important risk factor for pCMS. Patients with pCMS have adverse survival outcomes.

Acute nonpharmacological management for brain tumor patients and their caregiver

  Khusboo, Nitin Garg; BTSG and Awareness Foundation India

Background: A diagnosis of Brain Tumor has a catastrophic effect on patient and caregivers with acute psychological stress. Traditionally, this aspect is not addressed appropriately. The patient and family members are left to tackle this situation of their own. Psychological support at this stage of diagnosis would have a significant impact on their ability to manage this psychological stress.

Materials and Methods: This was an observational study conducted over period from November 2019 to January 2021 of providing acute real time psychological support in the form of Supportive Therapy, Crisis Intervention, Grief Counselling, Psychoeducation and pre and post-surgical counseling. The Neuro Rehab. Psychologist is an active member of neurosurgical team and has awareness about the disease and current clinical status. About 300 Brain Tumor patients and their care givers comprising newly diagnosed and those under treatment have been provided this care. This includes both admitted patients and those on follow-up in out-patient services.

Finding: A significant improvement was found in hopelessness, helplessness, worthlessness and psychological distress of the patients. Care givers noticed improvements in their stress levels and their ability to tackle this situation better.

Conclusion: We conclude that presence of Psychologist as an active member of Neurosurgical or Neuro-Oncology team improves the ability of patients and their care givers to face the psychological distress right from the time of diagnosis and during treatment.

Keywords: Acute, brain tumor, caregiver, management, nonpharmacological

A rare case of cystic intraparenchymal meningioma

Syed Khizar,   Sankararao,   Kiran,   Komal,   Shibu, T. Hegde; Narayana Hrudayalaya

Objectives: Case report of young male patient with a rare cystic intraparenchymal meningioma without dural attachment, which was preoperatively misdiagnosed as pilocytic astrocytoma based on MRI.

Materials and Methods: A 21-year-old male presented with right upper limb focal seizures and right grip weakness since 1year (80%power). On examination, he also had dyscalculia and updrift of right arm while checking for subtle signs of weakness. MRI-Brain showed solid-cystic lesion with mural nodule in left parietal lobe (?pilocytic astrocytoma). He underwent craniotomy and excision of the tumor. Postoperatively, he was discharged on POD3 with no fresh deficits. Postoperative MRI brain showed complete excision of tumor.

Results: Histopathology showed- tumor consists of meningothelial cells arranged in syncytial nests and lobules. The cystic wall is focally lined by proliferating meningothelial cells and thrown into folds. The wall shows a dense lymphomononuclear infiltrate, congested capillaries and hemosiderin deposits. Immunohistochemistry showed- vimentin and patchy EMA positive. GFAP, Synaptophysin, CK, Inhibin, L1CAM were negative. MIB-1 labeling ~5%. Features were suggestive of meningioma, WHO-Grade-I.

Conclusion: Intraparenchymal or subcortical meningiomas without dural attachment are extremely rare with only few reported cases. Till 2016, only 39 cases were reported in the literature. They are usually misdiagnosed preoperatively. According to their locations - classified into 5 varieties- intraventricular, deep Sylvain fissure, pineal region, intraparenchymal, or subcortical meningiomas and others.

They are more likely to occur in young males, with most common symptom of seizures. The most common location is the frontal lobe followed by the parietal lobe. They are more likely to be heterogeneous and cystic on imaging.

Some theories are proposed to explain the possible mechanism:

1) They arise from arachnoid cells of the pia mater, which enter the brain along with perforating blood vessels.

2) The meningiomas, which arise from the pia mater of brain sulcus, adheres and compresses the brain parenchyma, and grows into the intraparenchymal lesion, so the mass is seen to be completely buried in the parenchyma.

3) Some authors presume that the arachnoid cells rest during the migration progress.

4) Intraparenchymal meningiomas are believed to arise from ectopic meningothelial cells within the stroma of the pia mater.

5) The occurrence may be due to cellular dedifferentiation within the cerebral parenchyma, or they may arise from the sheath cells of cranial nerves.

Cystic meningiomas are also quite rare, accounting for 2%–4% of all intracranial meningiomas. They are more common in male pediatric patients than adults. Such meningiomas are usually difficult to differentiate from gliomas, intracranial abscesses, hemangioblastomas and metastatic lesions.

Current status of nanomedicine in glioblastoma multiforme: A systematic review

Urja Parekh, Hrishikesh Sarkar; NMIMS Sunandan Divatia School of Science

Background: With all the current therapeutic advancements, Glioblastoma multiforme (GBM) is still an incurable disease with a median overall survival of a mere 15 months. Owing to their small size, high surface-to-volume ratio and ability to cross the blood–brain barrier, nanoparticles are being employed in therapeutics, diagnostics and theranostics for GBM.

Objective: In this systematic review, we describe the basis and types of nanoparticles utilized for treatment of GBM and further evaluate the outcomes of clinical trials involving nanotherapy in patients with GBM.

Materials and Method: Ten clinical trials conducted between 2001 until 2020 were analyzed using the PRISMA 2009 guidelines. A total of 225 patients were further sub-grouped as either primary or recurrent GBM.

Results: The primary outcome of median overall survival (mOS) was 8.2 months for the pooled sample, 6.75 months for primary GBM and 9.7 months for recurrent GBM. The median KPS improved from 80 to 90 after therapy. Number of adverse events due to nanotherapy were n = 32 (14.2%). The results show that existing nanotherapeutic strategies were able to extend the survival as well as quality of life with an acceptable adverse event rate.

Conclusion: While nanotechnology has shown much potential in being a futuristic medicine to treat GBM, it is far from achieving superiority among other “second line” options. Possible reasons and future directions are discussed.