Treatment can be as simple as surgically removing the tumor using a small incision. In more serious cases, extensive surgery, aggressive chemotherapy, and radiation may be required. The course of therapy is generally determined by the tumor type or grade. Doctors use a pathological evaluation to show how fast the tumor is growing and to determine the tumor’s size and stage; in other words, they look at the tumor under a microscope to determine the extent and aggressiveness of the disease. Treatment can be somewhat less aggressive for tumors that are small, lower grade, and completely removed by surgery.
More aggressive therapy with chemotherapy and/or radiation is usually reserved for high-grade sarcomas that are larger and/or have spread to other parts of the body.
Desmoid tumors generally occur in the arms or legs, but can arise anywhere. Children with Gardner’s syndrome and familial polyposis coli are at higher risk of developing desmoid tumors.
Symptoms: Often present as a painless, slow growing mass.
Treatment: Surgery is the primary treatment for desmoid tumors. If the tumor cannot be completely removed or the tumor recurs, other treatment can be considered. Radiation may be used to limit local recurrence.
Outcome: The chance for recovery is excellent with the complete removal of the tumor. Factors that increase the risk of recurrence include diagnosis at a young age, aggressive features on pathology, incomplete removal of the tumor, and underlying Gardner’s syndrome or familial polyposis coli. It is recommended that children who have this type of tumor be evaluated for a specific mutation (change) in the APC gene. Some children with desmoid tumors may need to be screened for colon cancer.
Desmoplastic Small Round Cell Tumor (DSRCT)
Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive tumor. This cancer almost always arises in the abdomen, but can develop in other parts of the body. This cancer spreads locally to the lymph nodes and the lining of the abdomen. It can also spread to the lungs, liver, and bones.
Symptoms: Symptoms depend on the primary site and extent of disease when diagnosed. Patients with abdominal tumors often present with an enlarged abdomen which is painful. Sometimes they also have nausea, vomiting and/or weight loss.
Treatment: Desmoplastic small round cell tumors are often treated with chemotherapy, radiation, and surgery. It is important to surgically remove all of the disease to eliminate the cancer. Chemotherapy is sometimes used to shrink tumors prior to surgery.
Outcome: The overall prognosis (chance of recovery) for patients with DSRCT is poor – fewer than 20% of patients are expected to live more than 5 years after diagnosis. The chance for survival is higher if doctors are able to surgically remove all tumors.