Overview
Atypical teratoid/rhabdoid tumor (ATRT) is a rare and aggressive neoplasm predominantly affecting children, typically presenting intracranially with a dismal prognosis due to its rapid growth and resistance to therapy 1.Diagnosis
Imaging studies (MRI, CT) essential for initial detection and localization 1.
Histopathological examination required for definitive diagnosis, identifying characteristic rhabdoid cells 1.
Immunohistochemistry often confirms diagnosis by detecting markers like SMARCB1/INI1 loss 1.Management
Surgery: Primary treatment aimed at maximal resection 1.
Chemotherapy: Combination regimens often used, though specific protocols vary; examples include vincristine, cyclophosphamide, and doxorubicin 1.
Radiotherapy: Considered in specific cases, such as spinal ATRT in adults, though evidence is limited 1.Special Populations
Pediatrics: Primary focus of ATRT cases; treatment strategies centered around aggressive multimodal therapy 1.
Adults: Rare; case reports suggest radiotherapy may play a role in management, particularly for spinal involvement 1.Key Recommendations
Maximal surgical resection should be pursued whenever feasible for ATRT patients (Evidence: Expert opinion 1).
Multimodal therapy including chemotherapy is recommended, though specific regimens should be tailored based on institutional protocols and patient factors (Evidence: Expert opinion 1).
Radiotherapy may be considered in specific adult cases, particularly for spinal ATRT, given limited evidence (Evidence: Weak 1).References
1 Ayrak FB, Arslan SA, Tezcan Y. Spinal ATRT and Radiotherapy Case Report in an Adult Man. Oncology (Williston Park, N.Y.) 2023. link