Desmoplastic small round cell tumor
What is it? Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive tumor. This cancer almost always arises in the abdomen, but other primary sites have been described. This form of cancer spreads locally to lymph nodes and lining of the abdomen. It can also spread to the lungs, liver and bones. This cancer is more common in males and usually affects adolescents and young adults. The tumor has a characteristic genetic finding called a translocation. A portion of chromosome 11 is translocated to a portion of chromosome 22. Identifying this translocation is useful in confirming the diagnosis.What are the symptoms? Symptoms depend on the primary site of disease and the extent of disease at diagnosis. Patients with abdominal primaries often present with an enlarged abdomen and abdominal pain. They may also present with vomiting and weight loss.
How is it treated? Desmoplastic small round cell tumors are often treated with aggressive chemotherapy, radiation and surgery. It is important to surgically resect (remove) all sites of disease in order to eradicate the cancer. Unfortunately, this is often not possible. Using chemotherapy to shrink the disease prior to attempted resection is a reasonable approach. Some patients with DSRCT may benefit from more intensive therapy with autologous peripheral blood transplants, especially if they respond well to more standard therapy regimens.
What is the outcome? The overall prognosis for patients with DSRCTs is poor with less than 20% of patients expected to live more than 5 years after diagnosis. However, it depends largely on the ability to surgically remove all tumors.
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