|
|
|
|
Desmoplastic small round cell tumor
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 include the lymph nodes, the lining of the abdomen, and the pelvis. The most common site of spred is to the liver, lungs, and bones.
The tumor predominately strikes teenagers and young adults. It usually affects males, more than females.
There is no known risk factors that have been identified specific to the disease. Research has indicated that there is a relationship between desmoplastic small round cell tumor and the Ewings Family of Tumors.
Symtoms of disease include abdominal pain, abdominal mass, gastrointestinal obstruction, ascites, possible anemia, and cachexia.
At this time, the prognosis for desmoplastic small round cell tumor is poor, less than 20%. Usually, by the time the disease is diagnosed the tumor has already grown large within the abdomen and metastasized or seeded to other parts of the body.
There is treatment for the disease. Recent journals have reported that some patients respond to high dose chemotherapy, surgical debaulking, and radiation therapy.
This disease is also known as: Desmoplastic small round blue cell tumor; Intraabdominal desmoplastic small round blue cell tumor; Desmoplastic small cell tumor; DSRCT.
|
|
|
