Overview

Desmoplastic (des-moe-PLAS-tik) small round cell tumors are a rare and aggressive type of cancer that often starts in the belly. But it can happen in other parts of the body too.

Desmoplastic small round cell tumors (DSRCT) begin as a growth of cells. They often form on the tissue that lines the inside of the belly and pelvis. That tissue is called the peritoneum. The cancer cells can quickly spread to other organs, such as the liver, lungs and bones.

Desmoplastic small round cell tumors are a type of soft tissue sarcoma. Soft tissue sarcoma is a term used to describe a large group of cancers that all start in the tissues that connect, support and surround other body structures.

Desmoplastic small round cell tumors can happen to anyone. But they're more common in teen and young adult males.

Treatment for this cancer typically involves a few different options. Those options might include surgery, chemotherapy, radiation therapy and targeted therapy.

Symptoms

The symptoms of desmoplastic small round cell tumors depend on where the cancer begins. Most often it begins in the belly.

Signs and symptoms of desmoplastic small round cell tumors in the belly include:

  • Swelling in the belly.
  • Belly pain.
  • Constipation.
  • Nausea.
  • Problems urinating.
  • Weight loss.

When to see a doctor

Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you.

Causes

It's not clear what causes desmoplastic small round cell tumors.

In general, cancer begins when a cell develops changes in its DNA. A cell's DNA contains the instructions that tell a cell what to do. The changes tell the cell to multiply quickly. This creates a clump of cells called a tumor. The cancer cells can invade and destroy healthy body tissue. In time, the cancer cells may break away and spread to other parts of the body.

Risk factors

Healthcare professionals haven't found many risk factors for desmoplastic small round cell tumors. This cancer can affect anyone. But it's most common in males who are 15 to 35 years old.

By Mayo Clinic Staff