Desmoplastic Small Round Cell Tumor Treatment

Desmoplastic small round cell tumor (DSRCT) is a type of soft tissue sarcoma. It is a fast-growing cancer that grows in the body’s connective tissue.

DSRCT is most often found in the abdomen (belly). It can also be found in other parts of the body. Tumors are formed by small, round cancer cells. They are surrounded by scar-like tissue. DSRCT is rare. Fewer than 200 cases have been reported. It is most often found in white males ages 10–30. Males are about 4 times as likely to have DSRCT as females.

Desmoplastic small round cell tumor symptoms

Signs and symptoms of desmoplastic small round cell tumor include:

• Lump or swelling in the abdomen
• Belly pain or cramping
• Nausea
• Vomiting
• Diarrhea
• Constipation or trouble passing gas

Desmoplastic small round cell tumor diagnosis

DSRCT in the abdomen may not be found until the tumors have grown large. By then, the disease has often spread to the liver, lymph nodes, lungs, or bones.

Tests to diagnosis DSRCT may include:

• A health history, physical exam, and blood tests
• Imaging tests
• A biopsy of the tumor

The tumor has a specific gene change that helps confirm the diagnosis.

Desmoplastic small round cell tumor treatment

Because this tumor is so rare, there is no standard way to treat it. Treatments for DSRCT may include:

• Surgery is used to remove as much of the cancer as possible. Often, DSRCT has spread too far for complete removal.
• Chemotherapy (“chemo”) uses powerful medicines to kill cancer cells or stop them from growing (dividing) and making more cancer cells. Typically, 5 – 7 different types of chemotherapies are used to treat DSRCT.
• Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells or stop them from growing.
• Hyperthermic intraperitoneal chemotherapy (HIPEC) may be given during surgery. HIPEC kills cancer cells that cannot be removed surgically. It is done by washing the inside of the abdomen with a warm chemo solution. This procedure lasts up to 2 hours.
  

Desmoplastic small round cell tumor prognosis

The prognosis for DSRCT depends on where the tumor is located, if the cancer has spread, and how well it can be treated with surgery. DSRCT tumors that are outside the abdomen and have not spread to other organs respond better to treatment. DSRCT in the abdomen or DSRCT that has spread to other parts of the body does not respond as well.

The survival rate is about 15% survival at 5 years. Patients with DSRCT often experience relapse (the cancer comes back after being successfully treated).

Desmoplastic small round cell tumor care at       St. Jude  

• Patients with DSRCT treated on a phase 1 trial that evaluated a PARP inhibitor conducted at St. Jude had improvement in their disease.
• The follow-up PARP inhibitor study, called ONITT, is now enrolling patients with relapsed or refractory disease. ONITT is a phase 1 / 2 study of a PARP inhibitor, onivyde, along with talazoparib or temozolomide in children and young adults with recurrent or refractory solid tumors and Ewing sarcoma.
• St. Jude offers a dedicated team of specialists to meet the needs of children with desmoplastic small round cell tumor, including:
  • Surgeons, doctors, and nurses who treat this cancer
  • Doctors who specialize in radiation therapy and pathology (making a diagnosis by looking at tumor tissue under the microscope)
  • Experts in diagnostic imaging and nuclear medicine
  • Genetic counselors
  • Clinical dietitians
  • Child life specialists
  • Psychologists
  • Researchers and scientists

More reasons to choose St. Jude for care include:

• We are consistently ranked among the best childhood cancer centers in the nation by US News & World Report.
• At St. Jude, we have created an environment where children can be children and families can be together.
• St. Jude patients and families never pay for services authorized by St. Jude.
• We lead more clinical trials for childhood cancer than any other hospital in the U.S. 
• St. Jude is the only National Cancer Institute–designated Comprehensive Cancer Center just for children. A Comprehensive Cancer Center meets rigorous standards for research that develops new and better approaches to prevent, diagnose, and treat cancer.
• The nurse-to-patient ratio at St. Jude is about 1:3 in hematology and oncology and 1:1 in the Intensive Care Unit.
• Patients may be able to get expert, compassionate care and treatment closer to their homes through the St. Jude Affiliate Program.

Desmoplastic small round cell tumor clinical trials

St. Jude offers clinical trials and cancer research studies for children, teens, and young adults with desmoplastic small round cell tumor.  

Learn more about clinical research at St. Jude.