DSRCTs

1 What are Desmoplastic Small Round Cell Tumors?

Desmoplastic small round cell tumors are a type of soft tissue cancer that mostly begin in the abdomen. This type of cancer is rare and mostly affects boys and young men.

These tumora can be aggressive and tend to form multiple tumors in the peritoneumand pelvis.

Desmoplastic small round cell tumors can rapidly spread to other organs of the abdominal cavity. Treatment usually involves combination treatment, such as surgery, chemotherapy and radiation therapy.

2 Symptoms

Symptoms of desmoplastic small round cell tumor include:

  • Abdominal mass
  • Abdominal and back pain
  • Abdominal distension
  • Loss of appetite and weight
  • Abdominal distension
  • Gastrointestinal obstruction

3 Causes

The exact cause and mechanism of desmoplastic small round cell tumors formation, is unknown.

It is associated with a unique chromosomal translocation t(11;22) (p13:q12) resulting in an EWS/WT1 transcript that is diagnostic of this tumor. This transcript codes for a protein that acts as a transcriptional activator that fails to suppress tumor growth.

4 Making A Diagnosis

Tests and procedures that are used to diagnose desmoplatic small round cell tumors include:

  • Tissue biopsy for lab investigation
  • Imaging tests, such as MRI, CT scan, PET scan and ultrasound to view if the cancer has spread to other neighbouring structures

5 Treatment

Treatments of desmoplastic small round cell tumors include:

  • Surgery in which doctors remove all the cancerous tissue. Surgery is only used in cases where the tumor hasn't spread to surrounding tissue.
  • Chemotherapy-in this treatment method, medications are used to kill cancer cells. It is usually used before surgery to shrink the tumor and after surgery to kill the remaining cancer cells that might be left after surgery.
  • Radiation therapy- uses powerful energy beams, such as X-rays to kill cancer cells. It is mostly used after surgery to kill the remaining cancer cells.
  • Stem cell transplant to replace bone marrow with bone marrow stem cells. High dose chemotherapy is given before the stem cell infusion.

6 Risks and Complications

There is risk that Desmoplastic small round cell tumors can spread to the surrounding tissues such as:

  • spleen,
  • diaphragm,
  • liver,
  • intestines,
  • lungs,
  • uterus,
  • bladder
  • genitals.
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