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Purpose: Despite the lack of high-quality evidence, anti-epileptic drugs (AEDs) are very commonly prescribed for seizure prophylaxis in patients with brain tumors even without prior history of seizures. We sought to review the current practices of prescribing AEDs among neuro-oncology health-care professionals. Materials and Methods: An e-mail with online link to a 15-item questionnaire regarding AED prophylaxis was sent to members of major academic societies representing the Indian Neuro-Oncology Community to assess the prevalent patterns of practice. Results: The online survey was completed by 318 of 3320 health-care professionals that were mailed for an overall response rate of 9.6%. Majority (68.9%) of respondents were radiation oncologists followed by neurosurgeons (22.9%) and medical oncologists/neurologists (8.2%). The practice setting spanned across the health-care spectrum, and over 75% reported having ≥ 5-year of experience in managing patients with brain tumors. Even for seizure-naïve patients, 65.1% of respondents routinely prescribed prophylactic AEDs with significant variability in the duration of such prophylaxis. Levetiracetam was the most preferred AED (60%) followed by phenytoin (35%) in the survey. Most respondents believed that tumor type, histological grade, and anatomic location influenced the risk of seizures. Despite widespread use in clinical practice, only 27.3% of the respondents strongly believed that prophylactic AEDs significantly reduced the risk of seizures. Over 93% of respondents agreed that a large randomized controlled trial would better inform therapeutic decision-making regarding AED prophylaxis. Conclusions: This survey of the Indian Neuro-Oncology Community demonstrates widespread variability in the frequency and duration of AED prophylaxis in seizure-naive brain tumor patients in contemporary practice.

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Patients with malignant brain cancers such as glioblastoma and brain metastases (BM) represent a population with a large unmet medical need, and a multitude of drugs have failed over decades. The current treatment modalities include surgery, radiation, and chemotherapy; yet, the median survival of patients with gliomas and BM remains abysmally low at 15 months and 2–14 months, respectively. In addition, standard treatments cause debilitating motor and neurological deficits. The paucity of effective therapies, despite intense investigation over the past several decades, represents inherent challenges to treating brain cancer and the critical knowledge gap in understanding tumor sensitivity, drug delivery, and microenvironmental shifts. Recently, immunotherapy has shown tremendous efficacy in melanoma and other cancers but has yet to revolutionize the treatment of brain cancers. However, as immunotherapy holds the promise of specifically targeting and eliminating tumor cells while sparing normal brain cells, innovative methods for investigating immunotherapy for brain cancer are essential for optimizing patient response. In this review, we will summarize the key issues and how a systems biology approach can help decipher this complexity and lead to better understanding and therapeutic targeting of the brain cancers.

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Background: Updated 2016 WHO classification of central nervous system tumors has unified solitary fibrous tumor (SFT) and hemangiopericytoma (HPC) as a single entity (SFT/HPC), Grading from I to III, in view of common genetic alteration namely fusion of NAB2 and STAT6 genes. Aim: This study aims to evaluate the role of STAT6 immunohistochemical expression in a cohort of meningeal-based tumors and its correlation with other pathological parameters. Materials and Methods: Sixty-two meningeal-based tumors were studied including 40 HPC/SFT (1 Grade I, 29 Grade II and 10 Grade III), 14 meningiomas (2 Grade I, 10 Grade II, 2 anaplastic meningiomas), three meningeal sarcoma, and five cases with features suspicious for SFT/HPC. The expression pattern of STAT6 and other histological findings were noted. Results: Nuclear or nuclear + cytoplasmic STAT6 positivity was seen in 40/40SFT/HPC (100%), diffuse in 30 and focal in 10 cases. Only 1/14 cases of meningioma (7.1%) showed weak nuclear positivity. All cases of meningiomas and 2/3 cases of meningeal sarcoma showed cytoplasmic positivity. Of the five suspicious HPC cases, four showed diffuse strong nuclear STAT6 positivity, based on which they were confirmed as HPC. Interestingly, of the 40 STAT6 positive SFT/HPC, five showed epithelial differentiation (AE1/AE3 and/or epithelial membrane antigen [EMA]). CD34 was negative in 4/36 SFT/HPC. CD34 was focally positive in 3/5 suspicious HPC cases, two of which were focally EMA positive as well, and both cases showed nuclear STAT6 positivity. BCl2 was negative in 5/27 SFT/HPC, all of which were nuclear STAT6 positive. Conclusions: Nuclear expression of STAT6 can serve as a highly sensitive and specific adjunctive diagnostic marker for meningeal-based SFT/HPC, especially in cases with nonconventional histology and immunohistochemical profile, where meningioma or high-grade meningeal sarcoma forms a diagnostic dilemma.

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Introduction: Novel biological insights have led to consensus classification of medulloblastoma into four distinct molecular subgroups. It is widely accepted that medulloblastomas have a higher propensity for extraneuraxial metastases (ENM) compared to other primary brain tumors. We sought to review our own institutional experience of ENM from medulloblastoma. Materials and Methods: Patients with a histological diagnosis of medulloblastoma and ENM were identified from institutional neuro-oncology database. Patient-, disease-, and treatment-related details were retrieved by retrospective review of medical case records. Results: A total of 573 patients with medulloblastoma were registered from 2005 to 2016, of which 13 patients were detected with ENM. Median age of our study cohort was 17 years (range: 3–25 years) at index diagnosis. Ten (77%) patients had desmoplastic medulloblastoma, while 3 (23%) patients had large-cell/anaplastic histology. Five of 7 (71%) patients with known molecular subgroup affiliation belonged to sonic hedgehog subgroup, while the remaining two (29%) were subgroup 3 medulloblastoma. The median time to development of ENM was 13 months (range: 2–72 months) with common sites being bone (77%), bone marrow (62%), and lymph nodes (46%). Median overall survival after diagnosis of ENM was 2 months (range: 1–25 months) with 1-year survival probability of 23%. Patients with desmoplastic medulloblastoma had longer median overall survival compared to anaplastic histology (4.5 months vs. 1 month; P = 0.07). None of the other putative prognostic factors such as age at ENM, concurrent central nervous system involvement, visceral metastases, or time to relapse impacted overall survival. Conclusions: ENM from medulloblastoma, although rare, is frequently associated with desmoplastic histology. Bone, bone marrow, and lymph nodes are the most common sites of extraneuraxial involvement. Survival outcomes remain universally poor mandating the testing of newer agents and/or novel molecularly targeted therapies.

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While the management protocol for de novo glioblastoma is well established, the role of surgery in recurrent high-grade glioma (HGG) is not yet clear. In the light of recent developments in radio and chemotherapy modalities, it has become essential to recognize true disease progression from its mimics. This article discusses the recent literature on the challenges in identifying recurrence in HGG, modalities to diagnose, indications for surgery, and the outcomes in contemporary studies. The factors identified in prognostication have been discussed.

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Over the last few years, there has been a shift from a 100-year-old tradition in classifying and grading brain tumors. Whereas traditional teachings were based on the observation of individual cells and identification of distinct recognizable patterns formed by these cells, modern classification demands the incorporation of molecular markers into the traditional systems of grading, causing considerable disruption in conventional histopathological approaches. The objective of this article is to highlight the advancements in grading and classification, to document the evolution of glioma prognostication, nil and nil the major events that marked turning points in its history. This review article is an exhaustive review of the literature that summarizes the changes in glioma classification over the last 100 years – from the early macroscopic inspections done at autopsy in the late 19^th century to the first introductions of molecular markers by the World Health Organization. At this time of change, this article gives us a glimpse of how grading and classification has evolved as science has progressed, providing a glimpse into the future. Understanding the history of grading and classification is the key to better understanding the integrated histopathological and molecular approaches of today.

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Surgery for gliomas has been dogged by controversy for decades. The need for radical resections and the extent of such resections has been a subject of many debates. The all-elusive cure coupled with the complex biology of the disease feeds this pessimism. However, advances in technology and integration of adjuncts into the operating room, along with the evolution of specialist training in this field, has seen improving outcomes documented in the ever-increasing scientific literature. This commentary provides an overview of this evolution and the status of contemporary glioma surgery.

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Optic pathway gliomas (OPGs) are histological low grade but widely infiltrative tumors that arise along the optic nerves, optic chiasm, tracts, diencephalic structures, and white matter radiations to the visual cortex. When OPGs progress, management strategies may differ between pre-chiasmatic and chiasmatic lesions. Radiation therapy is often utilized as the primary treatment modality, but it is typically postponed till the age of 5–7 years because of the potential for late risks such as endocrinopathy, vasculopathy, and cognitive decline. No definitive studies establish the superiority of one specific chemotherapeutic agent over others. However, a favorable side effect profile is often opted for. Surgery is considered in patients with (1) single nerve is involved, (2) progressive and disfiguring proptosis, (3) blindness, and (4) significant mass effect or hydrocephalus. Encouragingly, diffusion-tensor imaging (DTI) has emerged as a useful tool for the assessment of white matter structures, including the visual pathways. We report an interim analysis of 14 patients with OPG, at our center, a tertiary referral center. We report that magnetic resonance imaging (MRI)-DTI studies provided additional information about visual fiber arrangement in relationship to the tumor that was not evident by conventional MRI methods.

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Glioblastoma is one of the most dreaded tumors of the central nervous system. The tumors show poor prognosis with patients showing a median survival of only 12–15 months despite all the treatment regimens. Recent studies have contributed a lot to our understanding of the pathogenesis of this disease and have suggested the presence of glioma stem cell (GSC) population as drivers of tumor growth. The GSCs are also considered responsible for tumor recurrence leading to treatment failure. It is thus increasingly becoming important to understand the biology of this tumor and effectively classify these tumors based on genetic mutations. Advancement in diagnosis to uncover the stages of tumor development and customize treatment modalities would increase chances for better responsiveness to various treatments and help us witness positive outcomes. These altogether could help in improving the survival of patients and their quality of life. An urgent need to find better ways of diagnosis could be achieved by combining our knowledge of basic science with advanced technologies. This review summarizes the past and present status of glioma biology, their classification and pathogenesis. It highlights the current pitfalls in clinical treatment and recent advances in diagnostics which could have clinical implications in near future.

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The aim of this study was to validate the modified Pignatti scoring system as a predictor of survival in patients with World Health Organization (WHO) grade II diffuse fibrillary astrocytomas (DFAs). In this retrospective cohort study, data were collected from 135 consecutive patients who underwent excision/biopsy of WHO grade II DFAs from January 2000 to December 2015. The effect of the following prognostic variables on overall survival (OS) and progression-free survival (PFS) was studied: Pignatti score (PS = 1–4, low versus high risk), location of tumor (lobar versus nonlobar), extent of resection (subtotal resection [STR] versus <STR), and presence of enhancement (yes versus no). The median age was 42 years (range = 13–81 years) with male to female ratio of 1.8. A total of 99 patients had taken radiation therapy and had a follow-up >1 year (median follow-up = 5.2 years, range = 1–16 years). In all, 80 patients (81%, PS = 1–2) were in the low-risk group. The median PFS in the low-risk group (PS = 1 and 2) was 120 months (95% confidence interval [CI] = 72, 128). The median PFS in the high-risk group (PS = 3 and 4) was 72 months (95% CI = 18, not applicable [NA]). The median OS in the low-risk group was significantly higher at 128 months (95% CI = 72, NA) as compared to 72 months (95% CI = 18, NA) in the high-risk group (P = .005). The modified PS can be used to prognosticate survival in patients with WHO grade II DFAs, with patients of the low-risk category having a better OS but not PFS.

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Introduction: Primary brain tumors of different histological types developing spontaneously together is a rare event; the incidence being 0.3% of all brain tumors. Acoustic schwannomas form the majority (about 70%–80%) of all cerebellopontine angle tumors. The most common association found is meningioma and acoustic schwannoma occurring in the same cerebellopontine angle region. Excluding phakomatoses, other neurocutaneous syndromes, and previous irradiation; the simultaneous occurrence of different primary brain tumors with acoustic schwannoma is rare. Methodology: We report the largest single institute case series, 9 patients of simultaneously occurring primary tumors with acoustic schwannomas over the last 25 years (1990–2015). All these patients were operated for both the tumors. The classification, pathogenesis, and surgical strategy of such tumors are discussed in light of current literature. Results: There were five males and four females in the study population aged from 30-60 years. There were five patients with spatially distant co-existing tumors. Transient facial paresis was noticed in three patients. They had significant improvement within six months. Conclusion: Simultaneously occurring tumors need appropriate planning and surgical strategy for ensuring good outcome and long-term prognosis.

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Background: Brain metastases are a major cause of mortality and morbidity in cancer patients and are seen as a terminal event in the natural course of disease. Whole-brain radiotherapy (WBRT) has remained the most commonly used treatment for multiple metastases. Although it provides symptomatic relief, the effects have low durability and local failure is common. Stereotactic radiosurgery (SRS) techniques such as Gamma Knife have been shown to be as effective as surgery in control of limited (1–3) metastases. Aim: The aim of this is to study the role of SRS boost after WBRT in patients of 1–3 brain metastases. Objective: (1) To compare the survival of patients with 1–3 brain metastases treated with WBRT with versus without SRS boost. (2) To compare the duration of functional independence (FI) and normal neurocognitive function (NNF) posttreatment in the patients belonging to the two groups. Materials and Methods: Twenty-six patients with 1–3 brain metastases received WBRT to a dose of 30 Gy in 10 fractions. Half the patients (13) were also given an SRS boost of 16–20 Gy by the Gamma Knife technique. All patients were followed up at twelve weekly intervals for a period of 9 months and assessed for survival, FI (Karnofsky Performance Status Score (KPS) >60%) and NNF (Hindi Mental Status Examination Score >24). Results: At 9 months, the median survival in the SRS boost group was 27 weeks compared to 22 weeks in the no boost group. The mean duration of FI and NNF was 24 and 12 weeks in the boost and nonboost groups, respectively. The differences between two groups were not statistically significant. Conclusions: Although results are not significant, a definite trend toward improvement in median survival, FI, and neurocognitive function in patients who received an SRS boost after WBRT is seen.

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Context: Most primary intracranial neoplasms occur as solitary lesions. Presence of multiple intracranial tumours with different histological origin in same individual or even collision tumours is exceedingly rare. Aims: With the advancement in neuroimaging techniques, more such synchronous tumours are being identified. The most common combination identified till date is meningioma associated with glioma. Our understanding of multiple, dissimilar intracranial tumours will gradually improve as more cases are identified, reported and studied. The explanations for the simultaneous occurrence of different primary intracranial tumours in the absence of phacomatoses or prior radiation exposure are at present hypothetical, and these tumours could be simply coincidental. Material and Methods: We present here a series of four cases who had multiple intracranial tumours of different histological origin and were managed successfully. None of our patients had received radiation in the past or had any family history of phakomatoses. Conclusions: Simultaneous occurrence of histologically different primary braintumours is rare, and its preoperative diagnosis is still challenging. Syndromic association should be ruled out in patients of histologically different multiple brain tumours. Most common primary brain tumour associated with other histologically different brain tumours is meningioma. Thorough clinical and imaging studies are mandatory in these cases to provide optimal treatment.

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Context: Due to increased incidence of late neurotoxicities associated with whole-brain radiotherapy (WBRT), the idea of treating limited brain metastasis (BM) (1–4) only with stereotactic radiosurgery (SRS) has become an interesting topic. Due to lack of SRS facilities, there is a major disagreement in the use of SRS in BMs. Aims: In our study, we tried to find out views and practice patterns of the Indian radiation oncologists (RO) on the use of SRS in BMs. Setting and Design: This is an internet-based, cross-sectional survey among ROs. Materials and Methods: Online questionnaires were sent to 411 RO through an online survey portal (SurveyMonkey®). Responses were analyzed. Results: Among which, 24.6% (101) responded completely. 39.6% were from teaching hospitals and 49.4% (48) were had <5 years of postspecialization experiences. About 38% (36) treats 30–100 BMs cases/month. Among the respondents who have SRS facilities (25.8%), only 26.1% use SRS routinely for BMs. Majorities (39.7%) have <3-year experiences. In oligometastases (1-4), 56.1% still practice WBRT only, though 57.6% feel that WBRT followed by SRS boost is the ideal treatment. 30.2% RO feels that local control is most important outcome determining factor for single metastasis, 40.9% RO feels the best modality is SRS alone. For treating >5 metastases, 92.1% ROs practice WBRT only. Conclusions: This is probably the first study on practice patterns of SRS in India if not the world. Major discordance exists between practice patterns and views on SRS use among RO in India. Majority do not practice SRS due to lack of facilities.

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Objective: Evaluation of effect of interval between surgery to adjuvant radiotherapy (Sx-ART) start and its early tolerance in glioma patients. Method: A retrospective study of 155 adult glioma patients (WHO Grade II-IV) who underwent adjuvant radiotherapy (ART) post-surgery with confirmed histopathology was done in our institution from Sept, 2012 to May,2017. Results were analysed in 3 parts: Sx-ART interval, ART schedule, ART course tolerance. Results: Sx-ART interval ranged from 1-8wks. Seventy-eight percent (121/155) patients started ART within 4wks of surgery, 30/155(19.4%) started within 2wks and 89/155 (57.4%) within 3wks of surgery. ART schedule was conventional fractionation (CF) in 121 (78%) while 34/155 (22%) patients received hypofractionated (HF) course. CF schedules varied between 54Gy-61Gy in 28-33 fractions and HF ranged 50Gy-55Gy in 20-25 fractions. Duration of ART ranged 24-50 days (median 41days). Sixty-five percent (101/155) patients completed ART <6wks and 147/155 (95%) <7wks. Seventy-eight percent (121/155) patients had concurrent Temozolomide (TMZ) including 28/34 (82%) HF patients. ART course interruption was recorded in 11/155 (7%) patients. Interruptions for Sx-ART interval <3wks & >3wks was 3 & 8 patients respectively. None had Grade 3/4 skin reactions or post-treatment scar infection/gaping at ART conclusion. Conclusion: Early start of ART in this analysis showed no difference in tolerance to radiotherapy, including patients receiving HF radiotherapy or concomitant temozolomide. Decrease in time interval from surgery to start of radiotherapy may improve compliance to radiotherapy and reduce overall duration of treatment time (ODT). Long term data of such approaches to reduce ODT and its effect on disease free and overall survival require future studies.

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Neurothekeomas are benign, predominantly cutaneous tumors originating from the nerve sheath seen mostly in the first three decades with predilection to female gender. This is the second case in world literature describing neurothekeoma in the cerebellopontine angle (CPA). This patient was a 27-year-old female who presented to us with right-sided CPA neurothekeoma. Intraoperatively, the tumor showed no obvious attachments from the surrounding nerves, arachnoid, and brain tissue, and gross total resection was achieved. The tumor cells were strongly positive for S100 and focally for glial fibrillary acidic protein and were negative for epithelial membrane antigen, cytokeratin, and synaptophysin. More literature inputs along with long-term follow-up data are needed for this rare entity to form a uniform treatment protocol.

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Introduction: Intracranial meningioma is one of the most common primary intracranial tumors. It constitutes 10%–15% of all brain neoplasm across the globe, whereas in India, their occurrence varies from 11.6% to 21%. These tumors are neuroectodermal in origin and are believed to arise from arachnoid cap cells. These tumors are mostly histologically benign with <0.2% showing metastasis. Total excision of tumor is the treatment of choice. Patients and Methods: The survey intended to study the incidence of meningioma among other intracranial tumors, the clinical profile of the patients, surgical outcomes, and long-term results. The study included all the cases of intracranial meningioma admitted in the Department of Neurosurgery, Rajendra Institute of Medical Sciences, Ranchi, between the time period of January 2012–December 2015. All patients, irrespective of age and sex, who were diagnosed of intracranial meningioma based on clinical data, radiological evidence, and histopathology, were included in the study. A total of 106 patients were included in the study. Results: The study revealed that the incidence of intracranial meningioma among other brain tumors is 14.45% and the disease shows a female preponderance. Meningiothelial meningioma is the most common histological variant reported during the study. Seizure and cranial nerve damage were the common complications observed among the patients during the follow-up study. Total excision of the tumor is the treatment of choice. Seventy-seven percent of the patients show good recovery with Glasgow Outcome Score of 5. Conclusion: Intracranial meningiomas are common intracranial tumors. They affect people in fifth decade of their life and show a female preponderance. They commonly present with symptoms such as seizure and headache. Meningothelial meningioma is the most common histological subtype. Total excision of the tumor mass has been found effective in preventing reoccurrence. Microsurgical techniques give good results with minimum mortality.

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