Desmoplastic Small Round Cell Tumors (DSRCT): Causes, Symptoms and Treatment Options

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.


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)


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.


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.

Desmoplastic small round cell tumors are a type of cancer that can be difficult to treat. While several treatment options are available, the best course of treatment will depend on the individual case. If you or someone you know has been diagnosed with DSRCT, it is important to discuss all available options with a medical professional.


What is Sarcoma?

Sarcoma is cancer that can develop in many body parts, including bone, muscle, fat, and connective tissue. There are two main types of Sarcoma, Bone and Soft tissue Sarcomas.

Soft tissue sarcomas can develop in soft tissues like fat, muscle, nerves, fibrous tissues, blood vessels, or deep skin tissues. They can be found in any part of the body.

Most of them start in the arms or legs. They can also be found in the trunk, head and neck area, internal organs, and the area in the back of the abdominal (belly) cavity (known as the retroperitoneum).  Sarcomas that most often start in the bones are osteosarcoma, chondrosarcoma, and Ewing sarcoma.

There are more than 50 types of soft sarcomas, and some are quite rare. 


If your doctor suspects you have sarcoma, they will do a diagnostic imagining. This includes magnetic resonance imaging (MRI), computed tomography (CT) scan, positron emission tomography (PET) scan, X-ray, and ultrasound.

A CT scan gives the doctor a detailed 3D view of the scanned area.

If they find that there might be cancer, they may do a positron emission tomography (PET) scan that can detect cancer that the CT scan could not by using radioactive sugar. Cancer cells will use sugar much faster than our normal body cells.

If the PET scan confirms what the scans show, the final step to confirming you have cancer is taking some tissue from the cancerous areas to test. This is called a biopsy.

Once they have all the information, they will determine your cancer stage. The stage of cancer will help your doctor decide how to treat you. Staging is done on a numerical scale of 1-4. The higher the number, the more it has grown.

Risk Factors

  • Family history
  • BRCA2 gene mutation
  • Li-Fraumeni syndrome
  • Carney-Stratakis syndrome
  • Hereditary retinoblastoma
  • Familial adenomatous polyposis (FAP) and Gardner’s syndrome
  • Neurofibromatoses


The general rule of thumb with cancers in your tissue (solid tumor cancers) is if they can remove that tissue, they will do that. There are some reasons they would not be able to remove the tissue. If the tissue is too large, they may need to shrink it by using radiation therapy, chemotherapy, or a combination of both. If cancer has spread to other parts of the body, then surgery is not always an option either.


After completing your therapy, you will follow up with your doctor every 3-6 months for the first 2-3 years. They may repeat your scans and blood work to ensure that the cancer is not returning or growing.

Your doctor might want to see you sooner if they think it is medically necessary.

Things to think about:

  • Always get a second opinion. Healthcare professionals are humans and can see things differently.
  • A biopsy is a gold standard for diagnosing solid tumor cancers. You should not just start therapy without having one.
  • If you are feeling ill, having nausea, or anything out of the ordinary during your treatment, let your treatment team know! They might be able to help you. Remember, they are trying to help you, not make you miserable.
  • The best way to fight cancer is to catch it early. So, see your doctor yearly for a physical and screen early, especially if you have risk factors. The slight inconvenience is worth it!



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