Rare Kidney Tumors in Children

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MSK Kids’ pediatric kidney tumor experts are exceptionally skilled at diagnosing and treating pediatric kidney tumors that doctors elsewhere may rarely, if ever, see. Our tools, treatments, and support services ensure that your child has the best chance of beating the disease.

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Rhabdoid Kidney Tumors

Rhabdoid tumors are a group of highly aggressive childhood cancers. They commonly occur in the brain and are known as atypical teratoid rhabdoid tumors (ATRT). Another common place they occur is the kidney, where they are referred to as malignant rhabdoid tumors of the kidney. However, rhabdoid tumors can occur anywhere in the body. They are quite rare and usually found before age II. Nearly all rhabdoid tumors are caused by a mutation in a gene called SMARCB1 (also called INI1).

At MSK Kids, we use high-dose chemotherapy to treat rhabdoid tumors, which we believe provides the best chance of a cure. Our unique treatment approach is based on our experience treating children with other aggressive solid tumors. In addition, we research the cause of rhabdoid tumors. We have found that a gene called PGBD5 may contribute to these cancers developing. We are studying the role of the drug tazemetostat (TAZVERIK™) to treat children with rhabdoid tumors if their first treatment doesn’t work.

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Renal Cell Carcinoma

Renal cell carcinomas are the most common type of kidney cancer in adults, however, they are quite rare in children and adolescents. They make up less than 5 percent of all pediatric kidney tumors. We offer surgery and treatments that target specific genetic mutations in children with renal cell carcinomas. We are also conducting clinical trials of more new medicines to treat renal cell carcinomas associated with a specific translocation, called TFE, which is seen in younger patients.

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Renal Medullary Carcinoma

Renal medullary carcinoma (RMC) is an extremely rare and aggressive kidney cancer. It is seen mostly in teens with sickle cell disease or a sickle cell gene. Similar to those rhabdoid tumors, these children also have a mutation in SMARCB1. At MSK Kids, we often combine surgery with chemotherapy or other targeted approaches to treat patients with RMC. We are also studying if the drug tazemetostat (TAZVERIK™) works for these tumors.

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Clear Cell Sarcoma of the Kidney

We discovered an important driver of clear cell carcinoma of the kidney (CCSK) called the BCOR protein. Our researchers are leaders in the field and have published many studies about how CCSK forms and behaves. We treat CCSK with surgery followed by chemotherapy.

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Congenital Mesoblastic Nephroma

Congenital mesoblastic nephroma (CMN) is a rare tumor most often seen in infants. It is the most common kidney tumor in newborns under 1 month old. In about 10 to 20 percent of cases, it can be detected before the baby is born. At MSK Kids, we treat most infants with CMN using surgery alone. For those who need other or additional treatment, we use a unique approach that includes the drug larotrectinib (VITRAKVI®). This medicine blocks a protein called NTRK and can treat inoperable CMN tumors caused by the NTRK3 and ETV6 genes fusing together. MSK researchers played a big role in the US Food and Drug Administration’s approval of larotrectinib.

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