An Association of Pleuropulmonary Blastoma and Cystic Nephroma: Possible Genetic Association

Author:

Soglio Dorothée Bouron-Dal1,Harvey Isabelle1,Yazbeck Salam2,Rypens Françoise3,Oligny Luc L.1,Fournet Jean-Christophe1

Affiliation:

1. Department of Pathology, Hôpital Sainte-Justine, 3175 Chemin de la Côte-Ste-Catherine, Montréal, Québec H3T 1C5, Canada

2. Department of Surgery, Hôpital Sainte-Justine, 3175 Chemin de la Côte-Ste-Catherine, Montréal, Québec H3T 1C5, Canada

3. Department of Radiology, Hôpital Sainte-Justine, 3175 Chemin de la Côte-Ste-Catherine, Montréal, Québec H3T 1C5, Canada

Abstract

The association of pleuropulmonary blastoma and cystic nephroma is an uncommon entity, with only 4 cases of such an association in the same patient described in English literature. We report a 5th histologically documented case in a 32-month-old boy. The boy underwent a pulmonary biopsy that showed a pleuropulmonary blastoma and a nephrectomy that showed a cystic nephroma. The pleuropulmonary mass showed an important regression with postbiopsy chemotherapy, allowing subsequent tumorectomy. To date very little is known about this rare entity, and a genetic link between these 2 tumors is hypothesized.

Publisher

SAGE Publications

Subject

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

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