Desmoplastic small-round-cell tumor
Desmoplastic small-round-cell tumor (DSRCT) is an aggressive and rare cancer that primarily occurs as masses in the abdomen. Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large intestine, small intestine, bladder, brain, lungs, testicles, ovaries, and the pelvis. Reported sites of metastatic spread include the liver, lungs, lymph nodes, brain, skull, and bones. It is characterized by the EWS-WT1 fusion protein.
| Desmoplastic small-round-cell tumor | |
|---|---|
| Other names | DSRCT |
| Micrograph of a desmoplastic small round cell tumor, showing the characteristic desmoplastic stroma and angulated nests of small round cells. H&E stain. | |
| Specialty | Oncology |
| Prognosis | Five-year survival rate 15% |
| Frequency | ~200 cases reported |
The tumor is classified as a soft tissue sarcoma and a small round blue cell tumor. It most often occurs in male children. The disease rarely occurs in females, but when it does the tumors can be mistaken for ovarian cancer.
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