Primary Desmoplastic Small round Cell Tumor of the Kidney: A Case Report in a 14-Year-Old Girl with Molecular Confirmation-Reference-Cited by-同舟云学术

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

Published:2007-08 Issue:4 Volume:10 Page:320-324
ISSN:1093-5266
Container-title:Pediatric and Developmental Pathology
language:en
Short-container-title:Pediatr Dev Pathol

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

Link

http://journals.sagepub.com/doi/pdf/10.2350/06-10-0177.1

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