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The first case of a DSRCT was recorded in 1989, and about 200 cases have been diagnosed since then. The disease is most common in Caucasian boys. Although researchers think a problem with the genes may cause DSRCT, this has not been proven.
Since DSRCT often is not diagnosed until the cancer is advanced, tumors grow large and spread through the lymph system or blood stream to other parts of the body. They can spread to many areas of the body, including the lymph nodes, lungs, bone and liver. Other types of cancer, including rhabdomyosarcoma, may be in the abdomen as well.
DSRCT is a dangerous type of cancer that often is resistant to treatment and frequently comes back after treatment.
In rare cases, DSRCT may be passed down from one generation to the next. Genetic counseling may be right for you. Visit our genetic testing page to learn more.
Desmoplastic small round cell tumors (DSRCT) usually have no early symptoms. In most cases, the first sign is a hard, round mass in the abdomen, which can grow very large before it is found. The child may feel pain in the abdomen. Because DSRCT is rare, it is often misdiagnosed by pediatricians and family doctors.
Desmoplastic small round cell tumor (DSRCT) symptoms include:
- Swelling (distention) of abdomen
- Back pain
- Gastrointestinal blockage
- Lack of appetite and weight loss
- Fluid in the abdomen (ascites)
- Thyroid or hormone problems
DSRCT is rare. If your child has any of these symptoms, they are most likely caused by something less serious. However, it’s a good idea to talk to your child’s doctor, as they may signal other health problems.
As part of one of the nation’s largest cancer centers, MD Anderson’s Children’s Cancer Hospital has more experience with desmoplastic small round cell tumors (DSRCT) than most other hospitals. We have the latest, most accurate technology to diagnose DSRCT, and our physicians are highly specialized.
Since desmoplastic small round cell tumors are rare, few pediatricians or family physicians are familiar with the disease. Even many oncologists (cancer doctors) have never treated a case of DSRCT.
An early and accurate diagnosis greatly increases the chances for successful treatment. But, DSRCT is often misdiagnosed as other types of abdominal tumors until it has spread to other areas of the body.
If your child has desmoplastic small round cell tumor symptoms, the doctor will examine your child and ask you questions about your child’s health and your family medical history.
One or more of the following diagnostic tests may be used to find out if your child has DSRCT and if it has spread. These tests also may be used to find out if treatment is working.
Imaging tests, which may include:
- CT or CAT (computed axial tomography) scans
- MRI (magnetic resonance imaging) scans
- PET (positron emission tomography) scans
A biopsy is almost always needed to diagnose DSRCT. This involves removing a small amount of cells from the tumor or fluid in the abdomen and looking at them with a microscope. One of these methods is usually used:
- Fine needle aspiration (FNA): A thin needle is inserted into the tumor to remove a small amount of tissue or fluid.
- Core biopsy: This is similar to FNA, but a thicker needle is used to remove small cylinder-shaped samples (cores).
- Surgical biopsy: Tissue is removed during an operation.
Getting a second opinion at Children’s Cancer Hospital
Desmoplastic small round cell tumors can be difficult to diagnose. The pathologists at Children’s Cancer Hospital are highly specialized in diagnosing complex cancers, including DSRCT. We welcome the opportunity to provide second opinions for DSRCT.
If you would like to get a second opinion at Children’s Cancer Hospital, call 844-565-2361 to make an appointment or request an appointment online.
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.
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 844-565-2361 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 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.
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 (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
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.
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.
This procedure is similar to standard embolization, except chemotherapy agents are injected.
Why choose MD Anderson for your desmoplastic small round cell tumor treatment?
A team of highly focused renowned physicians, including surgeons, oncologists and radiologists, as well as a specialized support staff of nurses, physician’s assistants and others follow your child from diagnosis through treatment.
DSRCT often is difficult to diagnose. The Children’s Cancer Hospital utilizes the most advanced technology and techniques to pinpoint the cancer. We are at the forefront of discovering new therapies to help manage DSRCT, including:
- HIPEC (hyperthermic peritoneal perfusion with chemotherapy) which was pioneered at Children’s Cancer hospital, combines surgery with heated chemotherapy for a safe and often effective treatment. It has been shown to extend survival time in many patients.
- Targeted therapies that help fight DSRCT at the cellular level
Treating the whole child
Children’s Cancer Hospital is designed just for children, with a full range of services and amenities that help make the child and family’s experience as comfortable as possible. We go beyond medical care to deliver a comprehensive experience that treats the whole child.
And, at Children’s Cancer Hospital, you’re surrounded by the strength of one of the nation’s top cancer centers.
MD Anderson is a big organization, but it’s welcoming and it makes you feel safe. Help was always available
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