atypical teratoid rhabdoid tumor


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.