Read more about this disease, some with Classification – Types – Signs and symptoms – Genetics – Pathophysiology – Diagnosis – Screening – Prevention – Treatment and management – Cures and much more, some including pictures and video when available.
Desmoplastic small round cell tumor is classified as a soft tissue sarcoma. It is an aggressive and rare tumor that primarily occurs as multiple masses in the abdomen. Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large intestine, small intestine, bladder, brain, lungs, testicles, ovaries, and the pelvis. Reported sites of metatastic spread include the liver, lungs, lymph nodes, brain, skull, and bones.
The tumor is considered a childhood cancer that predominantly strikes boys and young adults.
The disease rarely occurs in females, but when it does the tumors can be mistaken for ovarian cancer.[1]
There are no known risk factors that have been identified specific to the disease. The tumor appears to arise from the primitive cells of childhood, and is considered a childhood cancer.
Research has indicated that there is a chimeric relationship between desmoplastic small round cell tumor and Wilm’s tumor and Ewing’s sarcoma.
DSRCT is associated with a unique chromosomal translocation (t11;22)(p13:q12) resulting in a EWS/WTI transcript that is diagnostic of this tumor.[2] This transcript codes for a protein that acts as a transcriptional activator that fails to suppress tumor growth.
There are few early warning signs that a patient has a desmoplastic small round cell tumor. Patients are often young and healthy as the tumors grow and spread uninhibited within the abdominal cavity. These are rare tumors and symptoms are often misdiagnosed by family physicians. The abdominal masses can grow to enormous size before being noticed by the patient. The tumors can be felt as hard, round masses by palpating the abdomen.
First symptoms of the disease often include abdominal distention, abdominal mass, abdominal or back pain, gastrointestinal obstruction, lack of appetite, ascites, anemia, and/or cachexia.
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