Onkologie. 2010:4(6):362-367

Desmoplastic small round cell tumor

Viktor Maňásek, Renata Soumarová
Oddělení radioterapie a onkologie, Komplexní onkologické centrum Nový Jičín

Desmoplastic small round cell tumor (DSRCT) is a rare disease from sarcoma group family. Typically, there is an extensive infiltration of abdominal

cavity, peritoneal effusion with no evident primary origin of tumor. Prognosis is unfavourable with 5-years overall survival described

in 15 % of cases even though multimodal treatment strategy is performed. We introduce case report of 34-years old patient with DSRCT

treated in our Oncology center. Reasonable schema of differential diagnosis of soft-tissue expansion in abdominal cavity is mentioned and

we emphasize crucial role of molecular genetic analysis. We focus on therapeutic approaches of this rarely occuring disease.

Keywords: desmoplastic small round cell tumor, DSRCT, EWS-WT1, peritoneal effusion

Published: January 1, 2011  Show citation

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Maňásek V, Soumarová R. Desmoplastic small round cell tumor. Onkologie. 2010;4(6):362-367.
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