Desmoplastic Small Round Cell Tumors Treatment

MD Anderson's Children's Cancer Hospital is among the few cancer centers in the nation with extensive experience treating desmoplastic small round cell tumors, a rare and aggressive form of pediatric cancer.

Using the latest research, and backed by the most modern technology and techniques, Children's Cancer Hospital physicians customize your child's comprehensive course of treatment to address specific problems. Our goal is to offer the best chances for effective treatment with the least impact on your child's body.

Surgical expertise

Surgery is usually the first line of treatment for DSRCT. Like all operations, surgery for DSRCT is most successful when performed by a specialist with a great deal of experience in the particular procedure. Children's Cancer Hospital surgeons are among the most skilled and renowned in the world.

HIPEC, or hyperthermic peritoneal perfusion with chemotherapy, an innovative surgical procedure pioneered at Children's Cancer Hospital, has shown to be safe and effective for many children with DSRCT. We are one of the few hospitals in the world offering this therapy.

Adapted from an adult surgical procedure for abdominal tumors, HIPEC involves debulking, or surgically removing, as much of the tumor(s) as possible. Heated chemotherapy is then circulated in the abdomen.

Discovering innovative approaches

The specialists at Children's Cancer Hospital are researching new ways to treat DSRCT, including targeted therapies to help the body fight cancer on a cellular level. Clinical trials are available for new agents to treat this rare disease, many available only at Children's Cancer Hospital.

If your child has been diagnosed with DSRCT, we’re here to help. Call 877-632-6789 to make an appointment or request an appointment online.

Desmoplastic small round cell tumor treatments

After carefully evaluating your child’s case, our team of experts will discuss a recommended course of treatment for DSRCT. Since DSRCT is complex and seldom seen, no standard treatment exists.

Surgery

Surgery is almost always part of treatment for DSRCT. Procedures may include:

• Debulking surgery: Surgical removal of as much of the tumor or tumors as possible. Any remaining tumor cells are likely to spread to other parts of the body. The surgery is complex, and may involve several organs.
• Hyperthermic Intraperitoneal Chemotherapy (HIPEC): Pioneered at Children’s Cancer Hospital, this innovative surgery improves outcomes for some children. The procedure begins with a 10- to 12-hour debulking procedure. Then a heated chemotherapy is circulated in the abdominal cavity.

Chemotherapy

Some DSRCT patients respond to chemotherapy, but most relapse. Long-term, low-dose chemotherapy may help patients in remission or with a tumor that cannot be surgically removed.

Radiation therapy

Radiation therapy (also called radiotherapy) uses high-energy beams to destroy cancer cells. New radiation therapy techniques and remarkable skill allow Children’s Cancer Hospital doctors to target tumors more precisely, delivering the maximum amount of radiation with the least damage to healthy cells.

Stem cell transplant with Chemotherapy

Targeted Therapies

Children’s Cancer Hospital is leading into the future of DSRCT treatment by developing innovative targeted therapies. These agents are specially designed to treat each cancer’s specific genetic/molecular profile to help your child’s body fight the disease. The doctors who treat DSRCT at MD Anderson are dedicated researchers who have pioneered and actively lead national and international clinical trials with novel targeted agents.  

Radiofrequency ablation or cryoablation

Gammaknife® radiosurgery

Embolization

In this minimally invasive surgery, doctors inject material to stop blood flow to an area. By starving the tumor of blood, its growth can be slowed or stopped.

Chemoembolization

This procedure is similar to standard embolization, except chemotherapy agents are injected.