Patterns of care and survival outcomes in patients with an extraventricular neurocytoma: An individual patient data analysis of 201 cases

INTRODUCTION:: Extraventricular neurocytoma is a rare neuronal tumor arising outside the ventricles. However, because of its rarity, its optimum treatment remains undefined. MATERIALS AND METHODS: We intended to perform an individual patient data analysis to examine the patterns of care and prognostic factors involved in the treatment of extraventricular neurocytomas. PubMed, SCOPUS, and Google Scholar were searched with the following MeSH terms: "Neurocytoma, Extra ventricular neurocytoma, Spinal neurocytoma AND treatment, Survival" to find all possible publications pertaining to EVN. RESULTS: From 108 publications, we retrieved 201 patients of extraventricular neurocytoma. Their median age was 30 years (range: 0.6-78 years). Sixty seven patients were in the pediatric (age ≤20 years) age group. There was a bimodal age distribution. Surgical details were available for 132 cases, and 51.5% underwent a gross total resection whereas 41.7% underwent a subtotal resection. Adjuvant radiation was used in 40% cases. For the entire cohort, the median progression free survival was 77 months (53.3-100.7). However, we could not find an impact of any of the prognostic factors on survival. CONCLUSION: An extraventricular neurocytoma is a very rare disease with varied presentations and different sites of origin. Gross total resection remains the standard of care. Adjuvant radiation may be used for salvage. However, radiation therapy after subtotal resection of an atypical neurocytoma may be administered.