DESMOPLASTIC SMALL ROUND-CELL TUMOR

Desmoplastic small round cell tumor is an undifferentiated tumor assoc iated with serosal surfaces, especially the peritoneum, It is found pr edominantly in adolescents and young adults and is much more common in males than in females. The tumor has a characteristic histology, with extensive stromal tissue around islands of small and undifferentiated cells revealing the desmoplastic appearance, The coexpression of epit helial and mesenchymal antigens distinguishes this entity from other s mall round and blue cell tumors of this age group, Cytogenetic studies showed a t(11;22) translocation that differs from the Ewing's tumor t ranslocation and seems to be specific to this entity, The involvement of the WT1 and EWS genes in the translocation makes this tumor an inte resting subject for research on histogenesis and differentiation in sm all round and blue cell tumors. Copyright (C) 1996 by W.B. Saunders Co mpany