So we got the call today.....Atypical teratoid rhabdoid tumor (AT/RT) (cancer) is a rare tumor usually diagnosed in childhood. Although usually a brain tumor, AT/RT can occur anywhere in the central nervous system (CNS) including the spinal cord. About 60% will be in the posterior cranial fossa (particularly the cerebellum). One review estimated 52% posterior fossa, 39% sPNET (supratentorial primitive neuroectodermal tumors), 5% pineal, 2% spinal, and 2% multi-focal.[1] In the United States, three children per 1,000,000 or around 30 new AT/RT cases are diagnosed each year. AT/RT represents around 3% of pediatric cancers of the CNS.[2] Around 17% of all pediatric cancers involve the CNS; it is the most common childhood solid tumor.[citation needed] The survival rate for CNS tumors is around 60%; with AT/RT it is around 10%. Pediatric brain cancer is the second leading cause of childhood death, just after leukemia. Recent trends suggest that the rate of overall CNS tumor diagnosis is increasing by about 2.7% per year. As diagnostic techniques using genetic markers improve and are used more often, the proportion of AT/RT diagnoses is expected to increase. Because it is highly malignant, AT/RT has a high mortality rate. The two-year event-free survival for children under three is 11%, the overall survival rate is 17%. For children aged 3 years or older the event-free survival was 78% and the overall survival 89%.[3] Because most patients with AT/RT are less than three years old, the overall prognosis for AT/RT is very poor. Current research is focusing on using chemotherapy protocols that are effective against rhabdomyosarcoma in combination with surgery and radiation therapy.baby girl Mimi and pa-paw love you so much!! U are our world. And always will be.
Posted on: Thu, 03 Jul 2014 22:17:50 +0000
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