atypical teratoid rhabdoid tumor

A rare, highly malignant tumour of the paediatric brain and spinal cord.
Clinical findings Posterior fossa pressure (headache), vomiting, lethargy, and ataxia; spinal tumour present with progressive paraplegia.
Prevalence 3% of paediatric brain cancers (30/million/year, US).
Survival Two-year event-free survival, 11%; overall survival, 17%.
Prognosis Dismal.
Management Chemotherapy, as per rhabdomyosarcoma, combined with surgery and radiation.

References in periodicals archive

Atypical teratoid rhabdoid tumors are small blue cell tumors that often arise in the posterior fossa, where they can mimic medulloblastoma.

Atypical teratoid rhabdoid tumors of childhood: diagnosis, treatment and challenges.

Update on diagnostic practice tumors of the nervous system

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