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Atypical teratoid rhabdoid tumor (ATRT) is a highly malignant central nervous system tumor occurring almost exclusively in children. ATRT is classified as a malignant neoplasm of the central nervous system. Prevalence data are not certified in this packet. No causative genes are certified in this packet, and no inheritance pattern is documented.
Documented manifestations of ATRT include malignant neoplasm of the central nervous system, macrocephaly, hydrocephalus, seizures, muscle weakness, ataxia, nausea and vomiting, hemiplegia or hemiparesis, cranial nerve paralysis, cerebral palsy, cerebral calcification, migraine, reduced consciousness, limitation of joint mobility, irritability, and apathy. The tumor's location within the CNS influences the specific neurological deficits observed.
No causative genes or inheritance patterns are certified in this packet for atypical teratoid rhabdoid tumor. The tumor occurs predominantly in early childhood. Onset data are not certified in this packet.
ATRT is diagnosed as a malignant CNS neoplasm. Specific diagnostic criteria, imaging protocols, and pathological confirmation approaches are not certified in detail in this packet.
No foundational therapies or approved treatments for ATRT are certified in this packet. Active clinical trials include: NCT07087002 (Phase 1, Stanford University, recruiting) evaluating GPC2-CAR T cell therapy for relapsed or refractory CNS tumors; NCT02114229 (Phase 2, St. Jude Children's Research Hospital, active not recruiting) studying alisertib for rhabdoid tumors; NCT06622941 (Phase 2, Ono Pharmaceutical, recruiting) examining ONO-4538 in rhabdoid tumors; NCT07390539 (Phase 1, recruiting) evaluating B7-H3-directed CAR-T cell therapy in CNS neoplasms; and NCT06942039 (Early Phase 1, C17 Council, recruiting) piloting intrathecal topotecan with maintenance chemotherapy.
33 trials found
Prognosis data for atypical teratoid rhabdoid tumor are not certified in this packet.
Ten active or recruiting clinical trials are documented for ATRT in this packet, spanning Phase 1 through Phase 2 designs. Investigation areas include CAR-T cell immunotherapies targeting GPC2 and B7-H3, MEK pathway inhibitors, checkpoint inhibitors (NCT05286801, tiragolumab and atezolizumab), and locoregional CNS delivery approaches. NCT03155620 (NCI, Phase 2) applies targeted therapy directed by genetic testing.
Data assembled from 6 of 12 sources · Last updated Nov 4, 2026, 10:26 PM UTC
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Genetic and Rare Diseases Info Center
Common questions about atypical teratoid rhabdoid tumor
AI-curated news mentioning atypical teratoid rhabdoid tumor
Updated Sep 28, 2026
A systematic review has been conducted on teratoid Wilms tumor, compiling patient-level data from published literature. This review aims to enhance understanding and treatment approaches for this rare pediatric kidney cancer.
A case study highlights the use of hemispherotomy in treating drug-resistant epilepsy associated with atypical teratoid rhabdoid tumors. This surgical approach may offer new insights into managing complex cases of epilepsy linked to rare tumors.
A new report from the Pediatric Surgical Oncology Research Collaborative details outcomes and surgical management strategies for malignant rhabdoid tumor of the kidney. This research provides insights into treatment approaches for this rare pediatric cancer.