atypical teratoid rhabdoid tumor


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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 ?
Clinically, the distinction between atypical teratoid rhabdoid tumor and medulloblastoma is important because of the more aggressive nature of the former and the partially differing chemotherapy regimens.
This discussion illustrates that the collaboration between the oncologist and the pathologist has a critical role in appropriately classifying these primitive pediatric brain tumors to (1) identify prognostically important medulloblastoma variants, (2) separate atypical teratoid rhabdoid tumors from other small blue cell tumors, and (3) distinguish between central and peripheral PNETs when appropriate.
Atypical teratoid rhabdoid tumors of childhood: diagnosis, treatment and challenges.
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