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Summary Literature (0)
DOID:6785 - desmoplastic small round cell tumor


Disease Ontology 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.

Synonyms: desmoplastic small round cell tumor (morphologic abnormality), desmoplastic small round-cell neoplasm,

Xenbase Genes :



Other Model Organisms: AGR, MGI, ZFIN, FlyBase, WormBase, RGD

Ontology Viewers: Disease Ontology, EMBL-EBI, OLSVis tree view, Ontobee
Parent(s): sarcoma (is_a), small cell sarcoma (is_a)