Atypical Teratoid/Rhabdoid Tumor (ATRT): Historical Perspective, Pathology, Radiology, and Contemporary Clinical Advances

Abstract

Atypical teratoid/rhabdoid tumor (ATRT) is a rare, highly aggressive embryonal central nervous system (CNS) neoplasm of infancy and childhood. Since its recognition as a distinct entity in the late 20th century, breakthroughs in molecular pathology and neuroimaging have transformed diagnostic, prognostic, and therapeutic paradigms. This review synthesizes historical milestones, pathology and molecular biology, radiologic hallmarks, and advances of the past five years, with special emphasis on the WHO 2021 CNS tumor classification. We also highlight institutional experience at St. Louis Children’s Hospital (SLCH), where systematic data collection and volumetric analyses underscore the challenges and opportunities in modern ATRT care.