TREATMENT OUTCOME IN PATIENTS OF PRIMARY CENTRAL NERVOUS SYSTEM GERM CELL TUMOUR: CLINICAL EXPERIENCE FORM A REGIONAL CANCER CENTRE IN NORTH INDIA

Abstract

Primary intracranial germ cell tumour(GCT) is a rare entity and constitutes 2-3 % of all paediatric brain tumours. Clinical data of patients of primary CNS GCT treated at our institute from 2006-12 was collected by retrospective chart review. Overall survival(OS) was analyzed by Kaplan-Meier method. Twenty patients(M0-19,M3-1) met the study criterion (male:female = 7:3). Median age at presentation was 13 years. Tumour location was pineal in 10, suprasellar in 6, thalamic in 2, basal ganglion in 1 and spinal in 1 patient respectively. Surgical resection was gross-total in 7(35%), near-total in 2(10%), sub-total in 4(20%) and limited to biopsy in 6(30%) patients. Tumours were germinomatous, non-germinomatous and mixed GCT subtype in 17(85%), 2(10%) and 1(5%) patient respectively. Chemotherapy (median-4 cycles) was given in 19(95%) patients. The common regimens used were BEP in 14(70%) and EP in 5(25%) patients respectively (B-Bleomycin,E-Etoposide,P-Cisplatin). Radiotherapy (40-50 Gray, median-42 Gray) was delivered in 17(85%) patients- local radiation in 6 and whole ventricular, whole brain and craniospinal irradiation followed by boost in 5,3 and 3 patients respectively. After a median follow-up of 23.13 months (mean-32.36 months), 17(85%) patients were in complete response and 3(15%) patients had progressive disease. Death and disease recurrence were noted in 4(20%) and 1 patient respectively. Median OS was not reached. The actuarial rate of OS at 2 and 3 years were respectively 82.6% and 74.4%. Multimodality management comprising limited resection followed by platinum based chemotherapy and radiotherapy (40-50 Gray) is a reasonable treatment strategy in patients of primary CNS GCT at our institute.