What are Desmoplastic Small Round Cell Tumors?
Desmoplastic small round cell tumors (DSRCTs) are a rare and aggressive type of soft tissue sarcoma. The tumors typically develop in the abdomen and can spread to other body parts, such as the lungs, liver, and lymph nodes. DSRCTs are often difficult to diagnose and treat and have a poor prognosis.
This type of tumor is mostly found in White males between the ages of 10 and 30 years old.
Symptoms
The symptoms of Desmoplastic small round cell tumors (DSRCTs) can vary depending on the location and size of the tumor, but some common symptoms include:
- Abdominal pain or swelling
- Loss of appetite
- Fatigue
- Nausea and vomiting
- Constipation or diarrhea
- Abdominal mass or lump
- Blood in stool or urine
- Shortness of breath (if the tumor has spread to the lungs)
- Jaundice (if the tumor has spread to the liver)
- Swelling of the legs or arms (if the tumor has spread to the lymph nodes)
Diagnosis
DSRCTs are often challenging to diagnose because of how they appear on imaging. Ultimately, a diagnosis is made through a biopsy of the tumor tissue.
Imaging studies. Imaging tests such as CT or MRI scans can be used to see the presence of a tumor and to determine its size and location. These imaging studies can also help to determine if the tumor has spread to other parts of the body.
Biopsy. A biopsy is the removal of a small sample of tissue from the tumor for examination under a microscope to look for the presence of cancer cells.
Genetic testing. Genetic testing can be performed on the tumor tissue to detect the specific genetic changes that are seen with this type of cancer. This testing helps to confirm the diagnosis of DSRCT.
Treatment
The treatment of DSRCTs is often complex and can involve a combination of surgery, chemotherapy, and radiation therapy. Surgery is typically the first treatment used, and it is used to remove as much of the tumor as possible. However, due to the aggressive nature of the tumors, complete surgical removal may not be possible, and the tumors can come back after surgery.
Chemotherapy is often used to shrink tumors and help control the disease’s spread. Radiation therapy may also be used to shrink the tumors and help control the disease’s spread. However, due to the aggressive nature of the tumors and the poor prognosis, chemotherapy and radiation therapy are not always effective.