Disease Information

GTO ID(s) GTC2406 | GTC2696
Disease NameDesmoplastic Small Round Cell Tumor
SynonymsDO:desmoplastic small round-cell neoplasm
DefinitionDO: 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.
Xref NCI:C8300 | GARD:6265
Disease GroupCancer

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