Definition:
Also known as DSRCT, it is a very aggressive tumor that occurs rarely, a type of cancer that always grows in the abdomen and spreads locally to the lymph nodes. It can also spread to other parts of the body like the lungs, bones, and liver, chest walls, spinal cord, large and small intestine, skull, bladder, brain, ovaries, pelvis and testicles. This is most common in males especially in the young adolescent stage. This is a life threatening disease. This tumor is also called desmoplastic small round blue cell tumor, desmoplastic cancer, intraabdominal desmoplastic small round blue cell tumor or desmoplastic sarcoma.
Diagnosis:
Diagnosis of this disease is quite tricky. Through the use of CT scan and MRI, one can determine the extent of the tumor through its size of spread and its relationship to the body structures like bones and nodes that surrounds it. Some also detects the EWS/WTI gene fusion transcript as diagnosis.
Treatment:
There is no specific standard way to treat the disease and often times it is misdiagnosed as other illness. Once diagnosed, aggressive chemotherapy is applied and considered the most effective treatment for the tumor. Other treatments applied are debulking operation, radiation therapy, cytoreductive surgery. With other options as intensity-modulated radiation therapy, stereotactic body radiation therapy, clinical trials, intraperitoneal hyperthemic chemoperfusion, and hematopoietic stem cell transplantation.
Symptoms and Signs:
Symptoms vary depending on the location of the tumor and extent. Patient with tumors at the abdomen often have enlarged abdomen associated with pain, vomiting and lost of weight due to lack of appetite, abdominal mass, obstruction in the gastrointestinal area, and patients may become anemic.
Causes:
As of present time, there is no known cause of this disease. Although, it appears to grow out from primary cells of childhood.
:
desmoplasticsmallroundcelltumor
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