Desmoplastic Small Round Cell Tumors (DSRCT)

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What are desmoplastic small round cell tumors?

Desmoplastic small round cell tumors, or DSRCT, are tumors that grow in the abdomen and pelvic area of the body. Desmoplastic small round cell tumors are a type of soft tissue sarcoma, which is a type of cancer that forms in the connective tissue of the body. Connective tissues include fat, muscles, tendons, lymph and blood vessels, and nerves.

How common are desmoplastic small round cell tumors?

DSRCT is very rare. DSRCT occurs most often in young white males between the ages of 10 and 30. Some reports say that only about 200 cases of DSRCT have been recorded since the cancer was first described in 1989.

How are desmoplastic small round cell tumors diagnosed?

Many people with DSRCT do not have symptoms when the cancer first starts. Later, when the tumors get larger, symptoms can include:

Pain
Nausea and vomiting
Diarrhea
Constipation
Swelling in the abdomen

Imaging: If you have symptoms of DSRCT, your doctor will use imaging scans such as ultrasound, CT, MRI, and PET, to look at where the tumors are and how big they are. They will also check for signs that the tumor has spread to other parts of the body.

Biopsy: To check if the tumor is DSRCT your doctor will do a biopsy, taking a small sample from the tumor with a needle. An expert, called a pathologist, will study cells from the sample under the microscope to see what kind of tumor it is. Under a microscope, DSRCT cells look round. The pathologist might also check for a protein called EWS-WT1 that this type of tumor makes.

How are desmoplastic small round cell tumors treated?

There is currently no standard of care for DSRCT because it is so rare. However, there are some treatment options.

Surgery: Surgery is used to remove as much of the DSRCT as possible. If some cancer cells are left behind, the cancer can come back in the same spot or a different part of the body.

Chemotherapy: When the tumors are large, or the cancer cells have spread to other parts of the body, chemotherapy is used along with surgery.

Hyperthermic intraperitoneal chemotherapy (HIPEC): This treatment washes the inside of the abdomen with warm chemotherapy drugs. This procedure lasts about two hours. HIPEC can be done along with surgery. The chemotherapy kills the tumor cells in the abdomen without exposing the rest of the body to the drugs, which can cause side effects.

Radiation Therapy: Radiation therapy uses radiation to kill the cancer cells. External radiation therapy comes from a machine that aims radiation at the tumors. For internal radiation therapy, seeds, ribbons, or capsules that contain a radiation source are placed in your body, in or near the tumor.

Do desmoplastic small round cell tumors run in families?

We do not know if it runs in families because there are so few people with DSRCT.

How do desmoplastic small round cell tumors form?

We know that in DSRCT, chromosomes (the structures in your cells that contain all of your genes) break apart and get put back together in the wrong way. This can cause cells to not function like they should. In DSRCT, a gene called EWS wrongly joins with a region called WT1. Doctors will look for this change in chromosomes to confirm that your cancer is DSRCT.

What is the prognosis for someone with desmoplastic small round cell tumors?

The estimate of how a disease will affect you long-term is called prognosis. Every person is different and prognosis will depend on many factors, such as

Where the tumor is in your body
If the cancer has spread to other parts of your body
How much of the tumor was taken out during surgery

If you want information on your prognosis, it is important to talk to your doctor. NCI also has resources to help you understand your prognosis. The five-year survival rate for DSRCT is 15%. Keep in mind that doctors estimate DSRCT survival rates by how groups of people with DSRCT have done in the past. Because there are so few people with DSRCT, these rates may not be very accurate.

It is very important to work with a team of experts as soon as possible after diagnosis with DSRCT to improve your chances of survival.