IMMUNOHISTOCHEMICAL DETECTION OF THE WILMS-TUMOR GENE WT1 IN DESMOPLASTIC SMALL ROUND-CELL TUMOR

The desmoplastic small round cell tumour (DSRCT) is a rare, highly mal ignant neoplasm usually presenting in the abdomen of adolescent males, A characteristic translocation between the Ewing's sarcoma gene on ch romosome 22 and the Wilms' tumour gene WT1 on chromosome 11 has been d escribed, producing a fusion gene with expression of the DNA binding a rea of WT1. Some Wilms' tumour antibodies recognize epitopes of this p art of the WT1 protein, All four cases of DSRCT examined showed strong staining of the tumours with an anti-WT1 antibody, suggesting this ma y be useful in the diagnosis of these tumours.