| Desmoplastic small-round-cell tumor | |
|---|---|
| Other names | DSRCT[1] |
 | |
| 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%[2] |
| Frequency | ~200 cases reported[2][3] |
Desmoplastic small-round-cell tumor (DSRCT) is an aggressive and rare cancer that primarily occurs as masses in the abdomen.[4] 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.
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.[5]
- ^ RESERVED, INSERM US14-- ALL RIGHTS. "Orphanet: Desmoplastic small round cell tumor". www.orpha.net. Retrieved 17 November 2019.
{{cite web}}: CS1 maint: numeric names: authors list (link) - ^ a b "Desmoplastic Small Round Cell Tumor". St. Jude Children's Research Hospital. Retrieved March 22, 2023.
- ^ "Desmoplastic Small Round Cell Tumors (DSRCT)". National Cancer Institute. 16 September 2019. Retrieved March 22, 2023.
- ^ Lee YS, Hsiao CH: Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical and molecular study of four patients. J Formos Med Assoc 2007; 106: 854–860.
- ^ Bland AE, Shah AA, Piscitelli JT, Bentley RC, Secord AA (2007). "Desmoplastic small round cell tumor masquerading as advanced ovarian cancer". International Journal of Gynecological Cancer. 18 (4): 847–50. doi:10.1111/j.1525-1438.2007.01110.x. PMID 18081791.