desmoplastic small round cell tumor

Term ID

DOID:6785

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. (2)

References

• GARD:6265
• ICDO:8806/3
• NCI:C8300

Ontology

Human Disease   ( DOID:6785 )