A new molecular variant of desmoplastic small round cell tumor: significance of WT1 immunostaining in this entity.

@article{Murphy2008ANM,
  title={A new molecular variant of desmoplastic small round cell tumor: significance of WT1 immunostaining in this entity.},
  author={Amanda J Murphy and Karen Suzanne Bishop and Carlos Ochoa Pereira and Susan Chilton-Macneill and Michael P Ho and Maria Zielenska and Paul Scott Thorner},
  journal={Human pathology},
  year={2008},
  volume={39 12},
  pages={1763-70}
}
Desmoplastic small round cell tumor is a rare aggressive neoplasm, often presenting in young adult males. Although the classic features are well described, considerable clinical, pathologic, and immunohistochemical variation has been reported. The defining feature is a reciprocal translocation, t(11;22)(p13;q12), which fuses EWS on chromosome 22 to WT1 on chromosome 11. WT1 immunohistochemistry is reportedly useful in distinguishing desmoplastic small round cell tumor from other tumors. Herein… CONTINUE READING