Atypical teratoid rhabdoid tumor

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Atypical teratoid rhabdoid tumor (AT/RT) 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 (PF), 39% sPNET (supratentorial primitive neuroectodermal tumors), 5% pineal, 2% spinal, and 2% multi-focal.

Diagnosis

The standard work-up for AT/RT includes:

• MRI Magnetic resonance imaging of the brain and spine
• LP (Lumbar puncture) to look for M1 disease
• CT (Computed tomography) of chest and abdomen to check for a tumor
• BMA (Bone Marrow Asperant) to check for bone tumors. Often a doctor will want perform a stem cell transplant
• BX Bone marrow biopsy
• Bone scan

External links

• The Rhabdoid Registry
• Hilden JM, Meerbaum S, Burger P et al. (2004-07-15). "Central Nervous System Atypical Teratoid/Rhabdoid Tumor: Results of Therapy in Children Enrolled in a Registry". Journal of Clinical Oncology. 22 (14): 2877–84. doi:10.1200/JCO.2004.07.073. Retrieved 2007-05-23. CS1 maint: Uses authors parameter (link) CS1 maint: Explicit use of et al. (link)
• Beigel J, Kalpana G, Knudsen E et al. (2002). "The role of INI and the SWI/SNF complex in the development of rhabdoid tumors: Meeting Summary from the workshop on childhood atypical teratoid rhabdoid tumors". Cancer Research 2002 (63:): 323–28.CS1 maint: Uses authors parameter (link) CS1 maint: Explicit use of et al. (link)
• Huret J, Sevenet N (2002). "Rhabdoid predisposition syndrome". Atlas of Genetics and Cytogenetics in Oncology and Haematology.