Desmoplastic Small Round Cell Tumor

**Semantic type:** Neoplastic Process

**Definition:** An aggressive malignant soft tissue neoplasm of uncertain differentiation. It is characterized by a recurrent chromosomal translocation t(11;22)(p13;q12) and the presence of small round cells in a desmoplastic stroma. It usually affects children and young adults. The most common site of involvement is the abdomen. Patients usually present with abdominal distention, pain, ascites, and a palpable abdominal mass. The prognosis is usually poor.

**Synonyms:** - DSRCT - Desmoplas. small round cell tumor - Desmoplastic Small Round-Cell Neoplasm - Desmoplastic Small Round-Cell Tumor - Desmoplastic small round cell tumor - Desmoplastic small round cell tumor - Polyphenotypic Small Round Cell Tumor - desmoplastic small round cell tumor