Rhabdoid Tumor: Causes, Symptoms, and Treatment
Medically reviewed by Harsha Moole, MD, Internal Medicine. Last reviewed: October 2, 2026.
What kind of doctor treats Rhabdoid Tumor?
A pediatric oncologist usually leads the care team for a rhabdoid tumor. This doctor specializes in treating cancer in children. A direct primary care or concierge doctor can be a first stop for an evaluation and can refer you to a specialist when needed.
What it is
A rhabdoid tumor is a rare, fast-growing cancer that most often starts in the kidney or brain of young children. The name comes from how the cells look under a microscope; early researchers thought they looked like muscle cells, even though the tumor does not come from muscle.
Symptoms
Symptoms depend on where the tumor grows. A tumor in the kidney may cause a lump in the belly, belly pain, blood in the urine, or a fever that does not go away. A tumor in the brain may cause headaches, morning vomiting, trouble with balance, or changes in behavior.
Causes and who gets it
Most rhabdoid tumors are linked to a change in a gene called SMARCB1. In some children, the change is in every cell of the body, which is called rhabdoid tumor predisposition syndrome. These tumors are very rare and almost always appear in children under age three.
How doctors diagnose it
Doctors start with a physical exam and imaging tests such as ultrasound, CT, or MRI scans to show where the tumor is and how big it is. The surest way to diagnose it is a biopsy, which removes a small piece of the tumor so a pathologist can test the cells for the gene change.
Treatment
Treatment usually involves surgery to remove the tumor when possible. Chemotherapy is often used, and radiation may be added depending on the child's age and where the tumor is. Care is planned by a team of specialists at a children's cancer center.
When to see a doctor
See a doctor if a child has a lump in the belly, ongoing belly pain, blood in the urine, or a fever that will not go away. Headaches with morning vomiting, balance problems, or big changes in behavior also need prompt attention.
How a direct primary care or concierge doctor fits in
A direct primary care doctor is paid a flat monthly fee instead of billing insurance for visits. A concierge doctor usually charges more and often works alongside insurance. Either type can help a family manage the everyday parts of care, such as well-child visits, watching for side effects between treatments, and answering questions quickly. They cannot treat cancer themselves, so they would refer the child to a pediatric oncologist and a children's cancer center.
Common questions
Is rhabdoid tumor the same as rhabdomyosarcoma?
No. Rhabdomyosarcoma is a cancer of muscle-like cells and is more common. A rhabdoid tumor is a separate disease with a different gene change and different treatment.
Can adults get rhabdoid tumors?
It is very rare, but adults can develop tumors with rhabdoid features, most often in the kidney or other soft tissues. Specialists usually guide care in these cases.
Should family members get genetic testing?
If testing shows the gene change is present in every cell of the child's body, other family members may be offered genetic testing. A genetic counselor can explain what the results mean.
Where can families find doctors who treat rare tumors?
Children's hospitals and cancer centers often have teams that treat rare childhood cancers. A directory like FindMyDirectDoctor.com can also help families find a primary care doctor who can coordinate referrals and everyday care.
Sources
- NIH Genetic and Rare Diseases Information Center - Rhabdoid tumor
- National Cancer Institute - Soft Tissue Sarcoma-Patient Version - NCI
Medical Disclaimer
This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.
