Rhabdoid Kidney Tumors: Treatment Options

Rhabdoid tumors are rare, cancerous growths that most often happen in very young children and can start in the kidney or other soft tissues.

This family of tumors can also occur in the brain or spinal cord, where it’s called an atypical teratoid rhabdoid tumor (ATRT). A malignant rhabdoid tumor (MRT) is a tumor that develops in the kidneys and soft tissues.

Many cases are linked to mutations in the gene SMARCB1 (also known as INI1, SNF5, and BAF47).

Symptoms of Malignant Rhabdoid Tumor

Symptoms of MRT can be subtle, especially in infants and young children. A noticeable lump or mass in the child’s belly (abdomen) is a common sign.

Fussiness or irritability may be the only outward change at first, and some children may have trouble urinating or blood in their urine. Families who notice these signs should talk with their child’s doctor sooner rather than later.

Diagnosis for Malignant Rhabdoid Tumor

Diagnosing an MRT involves confirming the tumor type and assessing whether it has spread. A thorough physical exam and medical history are part of the evaluation. 

• Imaging tests, such as a CT scan (computed tomography), help doctors see the tumor and where it is in the body.

• A biopsy (the removal of a small tissue sample) confirms the diagnosis and is often tested for changes in the SMARCB1 gene.

Depending on findings, additional tests may be recommended.

Rhabdoid Kidney Tumor Treatment Options

Treatment for rhabdoid kidney tumors often involves more than one type of therapy, and there is no single established standard of care; the care team will tailor a plan to the child’s situation.

Surgery

Surgery is often an early step. When possible, surgeons aim to remove as much of the tumor as they safely can, which may mean removing part or all of the kidney, depending on the tumor’s size and location.

Chemotherapy

Chemotherapy uses medications to treat cancer and may be very aggressive in this setting to target cancer cells that may remain after surgery. The timing and drugs used depend on the treatment plan developed by the child’s oncology team.

Radiation Therapy

Radiation therapy may be considered, depending on the child’s age and overall situation, to help treat areas where cancer cells remain or to shrink tumors. Decisions about radiation are individualized.

Stem Cell Transplantation

In some cases, very intensive chemotherapy (and sometimes radiation) can affect the body’s ability to produce healthy blood and immune cells.

A stem cell transplant may be discussed, in which healthy stem cells (blood-forming cells) previously collected from the child are returned after high-dose therapy to support recovery.

Supportive Care During Treatment 

Alongside cancer treatment, supportive care focuses on comfort and managing side effects. This includes support for therapy-related side effects, preventing and treating infections, and supporting the child’s quality of life during and after treatment.

If you are looking for advanced cancer treatment centers in Florida, consider ACTC. We provide personalized treatment plans for each patient based on an in-depth discussion of their needs.

If you've been diagnosed with a rhabdoid kidney tumor and need personalized treatment, call 352-345-4565 or book an appointment.