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   Table of Contents - Current issue
January-December 2018
Volume 1 | Issue 1
Page Nos. 1-55

Online since Wednesday, November 14, 2018

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Ushering in a new era: The IJNO p. 1
Dattatraya Muzumdar
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Indian society of neuro-oncology: Travails and triumphs p. 3
Rakesh Jalali, Pankaj Kumar Panda
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Glioma surgery: The art and science p. 6
Aliasgar V Moiyadi
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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Extent of resection in glioblastoma – Where to draw the line? p. 11
Raymond Sawaya
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Milestones in neuropathology: Bridging morphology with molecules p. 14
Vani Santosh
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Challenges in glioblastoma biology and implications in personalized therapy p. 17
Divya Kumari Singh, Abir Mondal, Anjali Shiras
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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Understanding the brain tumor microenvironment: Considerations to applying systems biology and immunotherapy p. 25
Tiffany M Juarez, Jose A Carrillo, Achal A Achrol, Matthew P Salomon, Diego M Marzese, James H Park, Nitin S Baliga, Santosh Kesari
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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Multiple primary intracranial tumors with diverse histological origin p. 34
Harish Naik, Vernon Velho
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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Clinicians view and practice pattern on the use of stereotactic radiosurgery in brain metastases: A survey among Indian radiation oncologists p. 40
Kazi Sazzad Manir, Anindya Mukherjee, Debanti Banerjee, Abhishek Basu, Swapnendu Basu, Sourav Guha
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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Extraneuraxial metastases from medulloblastoma: Single-institution outcome analyses p. 46
Tejpal Gupta, Archya Dasgupta, Sridhar Epari, Neelam Shirsat, Girish Chinnaswamy, Rahul Krishnatry, Godajayant Sastri, Rakesh Jalali
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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A rare cerebellopontine angle neurothekeoma p. 53
Kartik Manoj Multani, Anandh Balasubramaniam, M Sharath Kumar, Pooja Chavali
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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