Desmoplastic small-round-cell tumor

Common card · No. 465147

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, parotid gland, chest wall, skull, spinal cord, large intestine, small intestine, bladder, kidney, brain, pancreas, 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. 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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