Desmoplastic Small Round Cell Tumor (DSRCT)

What is desmoplastic small round tumor (DSRCT)?

Desmoplastic small round cell tumor (DSRCT) is a rare type of soft tissue sarcoma. In DSRCT, fast-growing tumors develop in your peritoneum — the membrane that lines the inside of your abdomen and your pelvis.

Anyone may develop DSRCT, but it typically affects men and people assigned male at birth (AMAB) between ages 20 and 30. Healthcare providers have treatments that can put the condition into remission, but it often comes back (recurs).

How rare is DSRCT?

DSRCT is a very rare condition, affecting about 1 in 1 billion people worldwide since researchers first identified it in 1989.

What are the symptoms of desmoplastic small round cell tumor?

You may not develop symptoms until tumors get large enough to affect your abdomen and digestive system. Common DSRCT symptoms include:

• Abdominal pain.
• Constipation.
• Diarrhea.
• Distended (swollen) belly.
• Nausea and vomiting.
• Unexplained weight loss.

Many symptoms, like constipation, diarrhea, nausea and vomiting, are common ones that happen for many different reasons and may not be signs of a serious illness. But consider talking to a healthcare provider if you have symptoms like these that get worse or last for more than a few days.

What is the cause of DSRCT?

Researchers know this condition happens when changes in certain chromosomes create an abnormal gene known as EWS-WT1. They don’t know what triggers the change.

How is desmoplastic small round cell tumor diagnosed?

Healthcare providers diagnose this condition by doing a combination of a physical examination, imaging tests and genetic tests:

• Physical examination to check for masses (lumps) in your belly.
• Imaging tests, including CT scans, MRI scans and/or ultrasound tests, to look for tumors in your stomach lining and other areas of your body.
• Genetic tests to look for the chromosomal change that causes DSRCT.

How are DSRCTs treated?

Healthcare providers may treat desmoplastic small round cell tumors with:

• Surgery to remove the tumor(s).
• HIPEC, a cancer treatment that involves circulating heated chemotherapy drugs in your abdominal cavity after surgery.
• Chemotherapy.
• Radiation therapy.

Treatment may put the condition into remission, meaning you don’t have symptoms and tests don’t find signs of disease. But remission often doesn’t last, and you’ll probably need new or different treatment for recurring tumors.

Regardless of treatment type, ask your healthcare team about palliative care. In palliative care, you receive medical, social and emotional support that may help you to cope with living with a serious illness.

What are treatment side effects?

Surgery, chemotherapy and radiation therapy cause different side effects. Surgery to remove DSRCTs may cause side effects that include a reaction to general anesthesia and bleeding. Common radiation and chemotherapy side effects are:

• Diarrhea.
• Fatigue.
• Nausea and vomiting.

What is the prognosis for DSRCT?

Your prognosis is what healthcare providers believe you can expect to happen after treatment. They base prognoses on information like tumor locations, whether tumors spread and how much of the tumor they were able to remove during surgery.

Because these are very rare tumors, there isn’t a lot of research that providers can evaluate to develop a prognosis. That makes it hard for them to say what you can expect, including how long you may live.

Right now, most survival rate data show between 15% and 38% of people with DSRCT were alive five years after diagnosis. That said, one analysis showed treatment cured a small percentage of people with metastatic DSRCT. That means tumors spread from the peritoneum to other areas of their bodies. That same analysis showed 60% of people treated before tumors spread were alive five years after diagnosis.

How do I take care of myself?

Desmoplastic small round cell tumors are rare and serious cancerous tumors that may come back within months or a year after treatment. It’s not easy to live knowing that cancer may come back. Here are some suggestions that help:

• Understand your prognosis: DSRCTs are rare, and you may have trouble finding accurate, up-to-date information about these tumors. Don’t hesitate to ask your healthcare team what you can expect. They know you and your situation and are your best resource for information.
• Ask about participating in clinical trials: Researchers are evaluating different ways to treat DSRCT.
• Consider cancer survivorship: You’re a survivor from the day you received your diagnosis and for the rest of your life. Talk to your healthcare team about programs for people with recurrent cancer.
• Find support: Ask your healthcare team about support groups for people with rare cancers. Spending time with others who are going through the same or similar experiences can make living with DSRCT a bit less lonely.

When should I see my healthcare provider?

DSCRT often comes back. Your healthcare provider may recommend that you have imaging tests, like CT scans, every three to six months to check for new tumors in the lining of your abdomen or other areas of your body.

What questions should I ask my healthcare provider?

You may have lots of questions about your situation. Take your time to process everything that you’re learning. Here are some questions to help you get started:

• How do you know this condition is causing my symptoms?
• Can treatment cure it?
• What are your treatment recommendations?
• What are the treatment side effects?
• Should I consider participating in a clinical trial?