Metadata | |
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ID | DOID:6785 |
Name | desmoplastic small round cell tumor |
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. 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 |
ICDO:8806/3 |
Subsets |
DO_cancer_slim DO_rare_slim NCIthesaurus |
Synonyms |
desmoplastic small round-cell neoplasm [EXACT] |
Parent Relationships |
is_a small cell sarcoma |