Renal Medullary Carcinoma, from the CureRays guide library.

Renal medullary carcinoma is a rare, aggressive kidney cancer that almost always occurs in young people with sickle cell trait, and it is treated mainly with platinum-based chemotherapy because surgery alone is rarely enough.

What it is.

Renal medullary carcinoma (RMC) is a rare and very aggressive cancer that begins in the center (the medulla) of the kidney. Its most striking feature is that it occurs almost exclusively in people with a sickle hemoglobinopathy — most often sickle cell trait, the usually-silent carrier state — and it tends to strike young people, including teenagers and young adults, more often male, and is more common in people of African descent. The tumor's cells uniformly lose a gene called SMARCB1, which pathologists use to confirm the diagnosis. RMC frequently causes blood in the urine, flank pain, or weight loss, and unfortunately it has often already spread by the time it is found. Because it grows and spreads so quickly and resists most ordinary treatments, care centers on prompt platinum-based chemotherapy, with surgery and radiation playing supporting roles.

How radiation treats it.

Radiation damages the DNA inside cancer cells so they can no longer divide. In renal medullary carcinoma, chemotherapy carries the main load, but radiation is a valuable supportive tool: focused techniques like SBRT can control an isolated area of spread, and targeted radiation can quickly relieve pain from a bone metastasis, improving quality of life. Modern image guidance keeps the dose on the tumor while sparing nearby organs. Radiation is painless, given over a short course of daily sessions, and leaves no radioactivity in the body.

The ways we can treat it.

Palliative external-beam radiation. Targeted radiation relieves pain and controls tumor at sites such as bone or lymph nodes, shaping the dose to the problem area to ease symptoms quickly.

Stereotactic body radiation (SBRT). Delivers a few high, focused doses to an isolated metastasis or a spot of progressing disease, controlling it without surgery.

Conformal / IGRT photon radiation. Image-guided, intensity-modulated beams treat tumor in or around the kidney while sparing the bowel, spinal cord, and remaining kidney tissue.

Questions we hear often.

Does sickle cell trait cause this cancer? Renal medullary carcinoma occurs almost only in people with a sickle hemoglobinopathy, usually sickle cell trait — the carrier state most people never notice. Having the trait is a risk factor, but the cancer is still extremely rare among those who carry it. Researchers are studying whether avoiding intense exertion and dehydration may lower the risk.

Why is chemotherapy used before or instead of surgery? RMC spreads early and fast, so by the time it is found it is usually no longer confined to the kidney. Surgery alone rarely controls it. Platinum-based chemotherapy treats the whole body and is the most effective backbone; surgery is added in selected cases, often after chemotherapy.

What can be done given how aggressive it is? Care is best delivered quickly at a center experienced with this rare cancer, using platinum-based chemotherapy with surgery and radiation in supporting roles. Because standard options are limited, clinical trials testing therapies aimed at SMARCB1 loss and immune-based combinations are an important and encouraged option.

This guide is informational only. It is not medical advice — please confirm anything here with your care team.