An Aggressive Diagnostically Challenging CNS Malignant Neoplasm: Atypical Teratoid /Rhabdoid Tumour - 3 years Institutional Study in a Tertiary Care Hospital

Introduction: Atypical teratoid/rhabdoid tumors (AT/RTs) are rare and aggressive pediatric malignant rhabdoid tumors (MRTs) that occur within the brain characterized by biallelic loss of function alterations of SMARCB1. Aim: The aim of this study is to find out the incidence of atypical teratoid/rhabdoid tumors (AT/RTs) for 3 years in our institute. Objective: To analyse the demographic clinical and histopathological features of Atypical teratoid/rhabdoid tumors (AT/RTs). Materials and Methods: It is a retrospective study conducted at Department of Neuro Pathology, Institute of Neurology and Neurosurgery, Madras Medical College / RGGGH, Chennai. 5 cases were reported from June 2016 to June 2019. Results: Four cases of AT/RT reported in our institute were under one year of age. One case presented with drop metastasis. The incidence of AT / RT among CNS tumors in our institute is 1.60% and incidence among embryonal tumors are 15%. Conclusion: Atypical teratoid/ rhabdoid tumor is a highly aggressive grade 4 tumor with poor prognosis. Five year survival rate is 14% and overall survival is 29%. So, it requires multimodality treatment approach.