Pediatric Atypical Teratoid Rhabdoid Tumor of Central Nervous System: A Case Series with Review of Literature

Atypical teratoid rhabdoid tumor (AT/RT) of the central nervous system (CNS) is the rare, aggressive malignant neoplasm of infancy and early childhood and relatively rare in adults. Patients with ATRT typically follow a dismal course. Because of its rarity and rapid course and poor prognosis, there has been no consensus as to the optimal treatment of this tumor. We herein report a series of nine cases collected from the year 2015 to 2021 in a regional tertiary care center in North India. We retrospectively collected histologically diagnosed ATRT cases and obtained demographic and clinical data from the databases. We retrieved the archived slides and tissue blocks for analysis and found nine diagnosed cases of ATRT. The median age of presentation was 3 years (ranging from 0.9 to 13 years) and showed male preponderance (male to female ratio of 2:1). The mean duration of symptoms was 3.5 months with headache and vomiting being the commonest symptoms. The tumors showed heterogenous immunohistochemical expression. Patients with AT/RT underwent multimodal treatment comprising surgical resection, radiotherapy, and chemotherapy based on the patient’s age and tumor site and its resectability. The mean overall survival was 15.1 months (range, 1.5–30 months). ATRT is a rare neoplasm with a highly variable clinical course and poor prognosis. It portends poor outcomes in spite of a multimodal approach to treatment; hence, there is a dire need to help combat this enigmatic tumor.