How many cases of DSRCT are there?
DSRCT is extremely rare. Fewer than 200 cases have been reported, about 85 percent of them in Caucasian people. Boys and young men are about four times as likely to have DSRCT as girls and young women. It is usually diagnosed in males between ages 10 and 30.
What does DSRCT stand for?
Desmoplastic small round cell tumor (DSRCT) is an aggressive malignant neoplasm that occurs in adolescents and young adults. This tumor can co-express epithelial, neuronal, and mesenchymal markers. Clinical manifestations are often related to widespread abdominal disease.
How is DSRCT diagnosed?
If you have symptoms of DSRCT, your doctor will use imaging scans such as ultrasound, CT, MRI, and PET, to look at where the tumors are and how big they are. They will also check for signs that the tumor has spread to other parts of the body.
What are the symptoms of DSRCT?
Symptoms of DSRCT can include abdominal pain, cramps, abdominal distention, a feeling of fullness and constipation. Patients often present with an abdominal mass. Patients typically receive imaging studies, including computed tomography (CT) and magnetic resonance imaging (MRI).
Is DSRCT hereditary?
The genetic change involved in DSRCT is acquired throughout a person’s lifetime and is not inherited .
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Desmoplastic Small Round Cell Tumor (DSRCT) by Fernanda Arnaldez, MD and David Loeb, MD, PhD. Also available in Chinese, Japanese, French, Italian and Spanish. Desmoplastic small round cell tumor (DSRCT) is an aggressive malignant neoplasm that occurs in adolescents and young adults.
What are the treatment options for DSRCT?
However, there are some treatment options. Surgery is used to remove as much of the DSRCT as possible. If some cancer cells are left behind, the cancer can come back in the same spot or a different part of the body. When the tumors are large, or the cancer cells have spread to other parts of the body, chemotherapy is used along with surgery.
What are the immunotherapy approaches to DSRCT in neuroblastoma?
Immunotherapy approaches to DSRCT are also under investigation. DSRCT cells express the disialoganglioside GD2 on their surface, and this antigen has been studied as a potential target for immunotherapy in neuroblastoma. 44 The utilization of monoclonal antibodies such as 3F8 and 8H9 to treat DSRCT has also been proposed.