Malignant Rhabdoid Tumor

Also known as: rhabdoid tumor, childhood rhabdoid tumor.

What is a malignant rhabdoid tumor?

A malignant rhabdoid tumor is a rare fast growing childhood cancer that commonly occurs in infants and young children (average age 15 months). It usually starts in the kidneys (but can start anywhere in the body in soft tissues; in the brain it's called atypical teratoid/rhabdoid tumor) and frequently spreads early.

What causes malignant rhabdoid tumor?

Some cases present in families, and abnormalities in a gene have also been described. It is unclear however what causes the defect to occur in the first place.

What are the signs and symptoms of malignant rhabdoid tumor?

Pain presenting as fussiness in infants with blood in the urine, a large mass in the abdomen and high blood pressure are common. If the brain is involved, abnormal function of extremities or changes in level of consciousness may be found.

What are malignant rhabdoid tumor care options?

Treatment for malignant rhabdoid tumor typically involves a multidisciplinary group of specialists. Surgery to remove the tumor, followed by an aggressive course of chemotherapy with or without radiation to rid the body of cancer (for brain tumors radiation therapy is the cornerstone of management).

Many infants who undergo this treatment may also need a stem cell transplant to restore healthy blood cells after the treatment.

Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: April 04, 2022 05:28 PM