Renal anaplastic sarcoma

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Renal anaplastic sarcoma is a very rare kidney tumor that mainly affects children and young adults, and it is more common in girls. By 2017 about 25 cases had been reported. Because it is so rare, doctors don’t fully understand its course, but it can spread to the lungs, liver, and bones. Some cases have been linked to changes in the DICER-1 gene.

Diagnosis may involve taking a small tissue sample (aspiration cytology) and imaging tests such as CT scans to help plan surgery. The condition can occur from infancy up to about 40 years old; about 60% of patients are female.

Patients usually present with an asymptomatic abdominal mass. The tumor tends to be on the right side. Under the microscope, the tumor shows many spindle-shaped cells and sometimes bizarre-looking cells; cartilage or bone tissue may be present, and cysts can be seen.

Because the disease is so rare, the best treatment is not fully known. It is typically treated by surgically removing the tumor. Radiation therapy and chemotherapy have also been used in some cases.

The tumor was first described in 2007, during a review of a large series of renal tumors.