Primary Desmoplastic Small round Cell Tumor of the Kidney: A Case Report in a 14-Year-Old Girl with Molecular Confirmation

• DOI: 10.2350/06-10-0177.1
• Publication Date: 2007-07-17T15:01:43Z
• Authors: Sophie Collardeau...; Dominique Ranchèr...; Olivier Delattre; Stelly Hoarau; Philippe Thiesse; Rémi Dubois; Christophe Bergeron; Frédérique Dijoud; Raymonde Bouvier
• Keywords: Desmoplastic small-round-cell tumor; Immunostaining; Neoplasm

We report a case of desmoplastic small round cell tumor (DSRCT) arising in the kidney 14-year-old female. The subject presented with gross hematuria. Medical imaging uncovered left renal mass without regional or metastatic extension. showed morphological, immunohistochemical, and molecular features DSRCT. Immunostaining revealed polyphenotypic differentiation. Molecular analysis detected fusion transcript resulting from t(11;22)(p13;q12) reciprocal translocation, which characterized this neoplasm. Desmoplastic is rare, aggressive neoplasm that mainly affects young males usually presents widespread abdominal serosal involvement. This unusual localization should lead one to consider differential diagnosis blue tumors kidney.