desmoplastic small round cell tumor (DSRCT)

Introduction

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)

More general terms

• soft tissue sarcoma (STS)

Additional terms

• chromosomal translocation t(11;22)(p13;q12)

Database

• OMIM: https://mirror.omim.org/entry/133450