Search
desmoplastic small round cell tumor (DSRCT)
Epideimiolpgy:
- predilection for young males
Pathology:
- abdominal serosal involvement
- poor prognosis
- a primitive histologic appearance
- classified as sarcoma by analogy to Ewing's sarcoma & similar translocations involving the EWS gene
Genetics:
- chromosomal translocation t(11;22)(p13;q12) involving WT1 with EWSR1 is associated with desmoplastic small round cell tumor (DSRCT)
Interactions
disease interactions
Related
chromosomal translocation t(11;22)(p13;q12)
General
soft tissue sarcoma (STS)
Database Correlations
OMIM 133450
References
OMIM :accession 133450