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Atypical teratoid rhabdoid tumor (AT/RT) is a highly malignant central nervous system tumor found almost exclusively in children, as stated in the condition definition. It is classified as a CNS rhabdoid neoplasm. Prevalence data are not established for this condition in available sources.
Clinical presentation involves malignant neoplasm of the central nervous system (observed in 80–99% of individuals), with irritability, apathy, and nausea and vomiting also very frequently reported. A range of neurological features—including macrocephaly, muscle weakness, seizures, hydrocephalus, ataxia, reduced consciousness, and hemiplegia or hemiparesis—each occur in approximately 30–79% of individuals. Cranial nerve paralysis, cerebral calcification, and cerebral palsy are occasional findings, present in approximately 5–29% of cases.
The causes of atypical teratoid rhabdoid tumor involve molecular alterations affecting CNS tissues that result in malignant tumor development. Specific genetic details and inheritance information are not characterized in this packet’s data fields.
Diagnosis involves neuroimaging and tissue sampling to confirm malignant CNS rhabdoid tumor histology. The condition typically presents in childhood. Clinical evaluation addresses the extent of CNS involvement, given the high frequency of neurological manifestations associated with this tumor type.
No treatments are listed as specifically approved for atypical teratoid rhabdoid tumor in this packet. Management of this highly malignant CNS tumor is complex and requires specialized pediatric oncology expertise. Several investigational agents have received orphan drug designation specifically for atypical teratoid rhabdoid tumor or related pediatric CNS tumor conditions.
33 trials found
The condition is described as highly malignant. Outcomes vary based on extent of disease and response to treatment. The frequent occurrence of features such as hydrocephalus, reduced consciousness, and hemiplegia reflects the serious nature of CNS involvement in this tumor. Individual outcomes depend on multiple factors, including extent of disease at presentation and access to specialized pediatric oncology care.
Atypical teratoid rhabdoid tumor is an active area of clinical investigation, with numerous ongoing clinical trials studying immunotherapy, targeted therapies, gene therapy approaches, and other interventions. Current information on trial enrollment is available through ClinicalTrials.gov.
Data assembled from 6 of 12 sources · Last updated Sep 20, 2026, 10:42 AM UTC
European rare disease database
Genetic and Rare Diseases Info Center
Common questions about atypical teratoid rhabdoid tumor
AI-curated news mentioning atypical teratoid rhabdoid tumor
Updated Apr 13, 2026
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.
A recent analysis from the National Cancer Database investigates the effects of radiation therapy on malignant rhabdoid tumors of the kidney. This study provides insights that could influence treatment protocols for this rare pediatric cancer.