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Metadata
ID DOID:6785
Name desmoplastic small round cell tumor
Definition A 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.
https://en.wikipedia.org/wiki/Desmoplastic_small-round-cell_tumor, https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-soft-tissue-tumors/desmoplastic-small-round-cell-tumors
Xrefs

GARD:6265

NCI:C8300

Subsets

DO_cancer_slim

Synonyms

desmoplastic small round-cell neoplasm [EXACT]

Parent Relationships

is_a sarcoma

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