A Typical Teratoid / Rhabdoid Tumor (ATRT) in a 6 Year Male: A Rare Case Report

Era Sankhyayan, Aashutosh Sharma

Abstract: Atypical teratoid /rhabdoid tumor (ATRT) is an extremely rare and highly malignant CNS (central nervous system) embryonal tumormost commonly seen in children less than 3 years of age. The most common location of its occurrence is cerebral hemisphere in posterior fossa. Radiological and histopathalogical features are similar to those of medulloblastoma and other CNS primitive neuroectodermal tumors (PNET). The key differentiating feature between ATRT, meduloblastoma other PNET is the presence of characteristic rhabdoid cells and typical molecular finding of deletion of INI1 on chromosome 22. Due to rarity and aggressive nature of tumor, there are yet no consensus guidelines regarding the management of this tumor. Prognosis is poor despite of treatment with high tendency of early relapses. We hereby present a case report of a 6 years boy with ATRT in supratentorial location in parietal lobe treated at our centre by surgical excision followed by adjuvant radiotherapy to cranio - spinal axis and a boost dose to tumor bed.

Keywords: Teratoid, Rhabdoid Tumor, ATRT

How to Cite?: Era Sankhyayan, Aashutosh Sharma, "A Typical Teratoid / Rhabdoid Tumor (ATRT) in a 6 Year Male: A Rare Case Report", Volume 12 Issue 7, July 2023, International Journal of Science and Research (IJSR), Pages: 2162-2165, https://www.ijsr.net/getabstract.php?paperid=SR23711001124, DOI: https://dx.doi.org/10.21275/SR23711001124