Term Name: | desmoplastic small round cell tumor |
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Synonyms: | desmoplastic small round-cell neoplasm |
Definition: | A small cell sarcoma that 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. |
Ontology: | Human Disease [DOID:6785] ( DOID:6785 ) |