Clear Cell Sarcoma

Clear cell sarcoma (CCS) is a very rare form of soft tissue sarcoma. It typically develops in the deep soft tissues that surround and support the tendons and muscles in your lower legs or feet. Experts estimate it affects 1 in 1 million people each year.

People may develop clear cell sarcoma at any age, but it most often affects teenagers and young adults in their 20s and 30s.

This disease causes slow-growing tumors. The tumors may move from your deep soft tissues to places just under your skin or other areas of your body. Clear cell sarcoma may spread to your lungs or lymphatic system.

Healthcare providers can treat this condition with surgery, radiation therapy and/or chemotherapy.

Clear cell sarcoma symptoms

The most common symptom is a lump under your skin on your leg or foot. It may not hurt, but it doesn’t go away and may get larger. Later, you may have more symptoms, including:

• Fatigue
• Night sweats
• Unexplained weight loss

Clear cell sarcoma causes

Researchers believe the accidental fusion of two genes causes clear cell sarcoma. Fusion genes or fused genes happen when genes from different chromosomes combine.

In this case, the EWSR1 and ATF1 genes combine to make the fused gene EWSR1/ATF1. The fused gene creates an abnormal protein that causes cells to multiply uncontrollably. Researchers don’t know why the EWSR1 and ATF1 genes combine. But they do know fusion takes place after you’re born.

How doctors diagnose this condition

A healthcare provider will ask about your symptoms, including when you first noticed them. They’ll do a physical exam and the following tests:

• MRI scan
• Biopsy to get tissue samples

A medical pathologist will examine the tissue samples for signs of the fusion gene EWSR1/ATF1.

What questions should I ask my healthcare provider?

Clear cell sarcoma is a rare disease. You may not know much about it. You may want to ask questions like:

• Where’s the tumor?
• Has it spread anywhere else in my body?
• Will I need surgery?
• What other treatments do you recommend?
• Will treatment cure it?
• Will clear cell sarcoma come back after treatment?

How is it treated?

Surgery followed by radiation therapy is the most common treatment.

• Your surgeon will remove the tumor and any lymph nodes in areas where the tumor has spread.
• You’ll have radiation therapy after surgery to kill any remaining cancer cells.

Medical researchers are testing immunotherapy medications and other anticancer medications to see if they keep clear cell sarcoma from spreading.

Recovery time

Your recovery from cancer surgery depends on factors like the tumor location, tumor size and whether the cancer has spread. Your care team will explain what you can expect.

Clear cell sarcoma can come back or develop in a new area of your body. It can come back many years after treatment. Your provider may recommend regular checkups, including MRIs, to look for signs of cancer.

When should I see my healthcare provider?

You should let your provider know about:

• Infection symptoms, like chills, fever, thick discharge from the surgery site or skin at the site that feels warm to the touch
• Changes in your body, like another lump that doesn’t go away

Is clear cell sarcoma curable?

Early diagnosis and treatment may cure clear cell sarcoma. But the disease may come back or develop in another area of your body. Your healthcare provider may recommend regular MRI scans to check for new tumors.

What’s the survival rate for clear cell sarcoma?

CCS is rare, which makes it hard to estimate survival rates. Experts often analyze different medical studies to make an estimate. One such study found 57% of people with CCS were alive five years after diagnosis.