What is rhabdoid tumor predisposition syndrome?

Rhabdoid tumors are abnormal growths that frequently affect the brain, kidneys or spinal cord, among other parts of the body.

The condition is related to mutations in the SMARCB1 or SMARCA4 genes. Individuals affected with rhabdoid tumor predisposition syndrome are more likely to grow these tumors than the general population. People with the disease may have some benign tumors but also do have an increased risk of cancer. In the brain the tumors are called ATRT- atypical teratoid rhabdoid tumors.

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Reviewed by: Ziad A Khatib, MD

This page was last updated on: 1/29/2019 3:21:13 PM