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Reports have indicated that patients will initially respond to first line chemotherapy and treatment but that relapse is common.
Some patients in remission or with inoperable tumor seem to benefit from long term low dose chemotherapy, turning DSRCT into a chronic disease.
Patients with advanced DSRCT may qualify to participate in clinical trials that are researching new drugs to treat the disease.
This disease is also known as: desmoplastic small round blue cell tumor; intraabdominal desmoplastic small round blue cell tumor; desmoplastic small cell tumor; desmoplastic cancer; desmoplastic sarcoma; DSRCT.
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.
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In older patients DSRCT can resemble lymphoma, peritoneal mesothelioma, and peritoneal carcinomatosis.
DSRCT is associated with a unique chromosomal translocation t(11;22)(p13:q12) Pathology reveals well circumscribed solid tumor nodules within a dense desmoplastic stroma. Tumor cells have hyperchromatic nuclei with increased nuclear/cytoplasmic ratio.
On immunohistochemistry, these cells have trilinear coexpression including the epithelial marker cytokeratin, the mesenchymal markers desmin and vimentin, and the neuronal marker neuron-specific enolase.
currently offers DSRCT patients the opportunity to send samples of their tumors free of charge for testing.
Research scientists are growing the samples on nude mice and testing various chemical agents to find which are most effective against the individual's tumor.