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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: 9/16/2025 12:13:51 PM

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Cardiac Tumor If an abnormal tissue grows in the heart it is known as a primary cardiac tumor.
Atypical Teratoid Rhabdoid Tumor An atypical teratoid rhabdoid tumor, is a fairly rare aggressive tumor usually diagnosed in young children that forms in the tissues of the Central Nervous System.
Rhabdoid Tumor Predisposition Syndrome Rhabdoid tumors are abnormal growths that frequently affect the brain, kidneys or spinal cord, among other parts of the body.
Radiation Therapy Radiation therapy is a common form of cancer treatment. It refers to exposing the body to beams of radiation that are used to kill cancer cells and prevent them from coming back.
Blood and Marrow Transplantation The transplantation of blood or bone marrow is the procedure of transferring and replacing the hematopietic stem cells.
HIPEC HIPEC is an abdominal cancer treatment that is used after all tumors have been surgically removed from the abdomen.
Chemotherapy Chemotherapy is a common form of cancer treatment. It refers to drugs that are often used to kill cancer cells and prevent them from coming back.