DOI: 10.36347/sjmcr.2014.v02i03.015

Pages: 161-164

Atypical teratoid/ rhabdoidtumour (ATRT) is a highly malignant neoplasm primarily affecting infants and young children. The majority of tumoursin the Central nervous system(CNS), approximately two-third arise in the posterior fossa. The histologic spectrum of AT/RT overlaps significantly with other forms of CNS neoplasms. The term "malignant rhabdoid tumor" was first used in reference to rare pediatric renal tumors. Neoplasms with such rhabdoid features have subsequently been identified in other locations, including the CNS with specific immunohistochemical and ultra structural features and consequently it has been called ATRT. On diagnostic imaging the tumor is most often diagnosed as medulloblastoma. The most common differential diagnosis are PNET and malignant glioma. Treatment planning depends on correct pathological diagnosis on squash cytology. FNAC or frozen section which acts as a primary method of diagnosis during intraoperative procedurelater confirmed on histopathology. Herein we present a case of a 10 year old male child who initially presented to the hospital with obstructive hydrocephalus, headache, vomiting and febrile illness.