Atypical Teratoid/Rhabdoid Tumors

Atypical teratoid/rhabdoid tumors (ATRTs) are rare embryonal brain tumors that are molecularly characterized by biallelic loss of function of the SMARCB1 gene or, in rare cases, SMARCA4. ATRTs predominantly affect young children, though rare cases have been documented in the adult population. Over the last two decades, significant progress has been made towards understanding the biology and clinical behavior of this disease. Herein, we review the current understanding of the histopathology, radiology, and clinical and molecular features of ATRT. We also provide an up-to-date summary of the advances in the treatment of ATRT.