Skip to Main Content
ALL DEFINITIONS

Rhabdoid Tumor Predisposition Syndrome

Definition

Rhabdoid tumor predisposition syndrome is a rare genetic condition characterized by an increased risk of developing aggressive rhabdoid tumors, which are fast-growing malignancies that can occur in various parts of the body, including the kidneys, brain, and soft tissues. This syndrome is caused by mutations in specific genes, such as SMARCB1 or SMARCA4, which play a role in regulating cell growth and division.