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

Author:

Collardeau-Frachon Sophie1,Ranchère-Vince Dominique2,Delattre Olivier3,Hoarau Stelly3,Thiesse Philippe4,Dubois Rémi5,Bergeron Christophe4,Dijoud Frédérique6,Bouvier Raymonde1

Affiliation:

1. Service d'Anatomie Pathologique, Hôpital Edouard Herriot, Lyon, France

2. Service d'Anatomie Pathologique, Centre Léon Bérard, Lyon, France

3. Unité de Génétique Somatique, Institut Curie, Paris, France

4. Service d'Oncologie Pédiatrique, Centre Léon Bérard, Lyon, France

5. Service de Chirurgie Pédiatrique, Hôpital Edouard Herriot, Lyon, France

6. Service d'Anatomie Pathologique, Hôpital Debrousse, Lyon, France

Abstract

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

Publisher

SAGE Publications

Subject

General Medicine,Pathology and Forensic Medicine,Pediatrics, Perinatology, and Child Health

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