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  • Medulloblastoma: Distinctive Histo-Molecular Correlation with Clinical Profile, Radiologic Characteristics and Surgical Outcome

    Final Number:
    830

    Authors:
    VINAYAK NARAYAN MD. M.Ch. DNB; Harsha Sugur M.Sc; Arivazhagan A; Vani Santosh; SAMPATH SOMANNA M.Ch

    Study Design:
    Other

    Subject Category:

    Meeting: Congress of Neurological Surgeons 2018 Annual Meeting - Late Breaking Science

    Introduction: Medulloblastoma[MB] is a heterogenous tumor and the prognosis is influenced by various clinical, histological and molecular factors. The aim of the study is to determine the clinical profile and radiologic characteristics among the histo-molecular subgroups,the predictors of surgical outcome and the pattern of relapse in pediatric and adult MB.

    Methods: An analysis of 118 patients of MB who underwent surgical treatment at National Institute of Mental Health and Neurosciences [NIMHANS],India over seven-year period is presented.The clinical profile, radiologic characteristics, surgical nuances and survival patterns are discussed.

    Results: The mean age of the cohort was 12 years.The primary manifestation was raised ICP headache in 53 patients[44.9%] which was the predominant symptom in LCA and WNT subgroups.The median pre-operative KPS was 60.Post-operative cerebellar mutism was noted in 13 patients [11%],unilateral abducent nerve palsy in 12 patients [10.2%],unilateral occulomotor nerve palsy in 10 [8.5%] patients,meningitis in 7 [5.9%] patients,seizures in 6 [5.1%] patients and operative site CSF leak in 4 patients[3.4%].Vermian and hemispheric location of tumor was most commonly observed in Non WNT/Non SHH [91.7%] and SHH [42.9%] subgroups respectively.92patients [78%] underwent pre-operative ventriculo-peritoneal shunts[VPS] for obstructive hydrocephalus and 14 patients[ 11.8%] underwent VPS in the post-operative period. The median OS for the whole group was 82.1 months and the median RFS was 51.0 months.While radiotherapy had a significant influence on overall survival, progression free survival was influenced by radiotherapy as well as chemotherapy in both pediatric and adult cohort. Desmoplastic and WNT tumors had the best prognosis;LCA and Non WNT/Non SHH had the worst prognosis.

    Conclusions: Pediatric MB comprised 82.2% of whole cohort and they had poor survival when compared to adults.Age, hemispheric location of tumor,extent of resection and adjuvant treatment status were the important clinical prognostic factors for survival.Surgery for MB is formidable and VPS can be considered in persistant symptomatic and progressive hydrocephalus.Our study on pediatric and adult MB validates the prognostic significance of various clinical,radiologic and histo-molecular parameters of MB.

    Patient Care: The research provide an in-depth view of clinical profile and treatment outcome in pediatric and adult MB. Also it demonstrates the various prognostic factors based on clinical, radiological and histo-molecular parameters.

    Learning Objectives: 1. Histomolecular correlation of medulloblastoma with surgical outcome. 2. Prognostic factors in pediatric and adult medulloblastoma. 3. Clinical profile variations in pediatric and adult cohorts.

    References: 1. Ellison DW, Dalton J, Kocak M, Nicholson SL, Fraga C, Neale G, et al. Medulloblastoma: clinicopathological correlates of SHH, WNT, and non-SHH/WNT molecular subgroups. Acta Neuropathol. 2011;121:381–96. 2. Kool M, Korshunov A, Remke M, Jones DTW, Schlanstein M, Northcott PA, et al. Molecular subgroups of medulloblastoma: an international meta-analysis of transcriptome, genetic aberrations, and clinical data of WNT, SHH, Group 3, and Group 4 medulloblastomas. Acta Neuropathol. 2012;123:473–84. 3. Kaur K, Kakkar A, Kumar A, Mallick S, Julka PK, Gupta D, et al. Integrating Molecular Subclassification of Medulloblastomas into Routine Clinical Practice: A Simplified Approach. Brain Pathol. 2016;26(3):334-43.

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