FB2025_05 , released December 11, 2025
FB2025_05 , released December 11, 2025
CV Term Report
Help Close All Open All
General Information
Term desmoplastic small round cell tumor ID (Ontology) DOID:6785 (Human Disease)
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.
Also Known As "desmoplastic small round-cell neoplasm"
Comment
Links to External Ontologies
DO.org
Annotations
Records annotated with this term OR any of its CHILD TERMS
No relevant records available
Records annotated with this exact term (annotations to child terms are NOT included)
No relevant records available
show Full annotation statements including this term (annotations to child terms are NOT included), and relevant FlyBase records
No relevant statements available
Spanning Tree (Parents/Children)
Only view relationship: 
  sarcoma
   |__small cell sarcoma
       |__desmoplastic small round cell tumor
Spanning Tree View Settings
Parents/Children
View Depth
Show hierarchy levels: for parents, for children
hide Relationships
Is a small cell sarcoma
Part of
hide Synonyms & Secondary IDs
Synonyms
  • "desmoplastic small round-cell neoplasm" EXACT
Secondary IDs
hide External Crossreferences & Linkouts
GARD:6265
ICDO:8806/3
NCI:C8300