Desmoplastic small round cell tumor (DSRCT) is a type of soft-tissue sarcoma that usually develops in the abdomen, but it can form in other parts of the body, as well (including the liver, spleen, lymph nodes, spinal cord, large or small intestine, lungs, pelvis, testicles, or bladder).
It is classified as a rare but aggressive childhood cancer that usually affects young men. In the rare instance that this type of mass develops in a female, it is often mistaken for ovarian cancer.
Signs & Symptoms
Since this type of cancer appears mainly in young children, symptoms often go unnoticed until the tumor has advanced considerably. The mass is usually quite large before the patient or their physician notices anything unusual.
The mass feels like a hard, round growth once it reaches a certain size. Symptoms can include distended abdomen, abdominal or back pain, loss of appetite, gastrointestinal distress, or anemia.
Diagnosis & Treatment
DSRCT is often misdiagnosed and mistaken for other conditions. Adults who suspect this type of mass should seek medical attention immediately. This type of tumor is very aggressive and spreads quickly. Patients should be seen by an oncologist or sarcoma specialist.
This type of cancer is very difficult to treat because of its aggressive, rapid-spreading nature. It may respond well to high doses of chemotherapy, and intense radiation. Some patients have undergone other aggressive procedures, including radiation of varying intensity and experimental clinical trials.
Prognosis
Most DSRCT patients have a very poor prognosis due to the aggressive nature of the disease; the 5-year survival rate is 15 percent. A patient’s prognosis depends on the stage of the illness; however, since it is often misdiagnosed, the mass may be quite large or may have metastasized (spread) to other parts of the body before it is properly diagnosed.
Some patients have relied on multiple forms of treatment to improve their survival odds; commonly chemotherapy, high doses of radiation, stem cell transplants, and surgery are all used in varying combinations to improve the patient’s quality of life and shrink (if not kill) the mass.
