胸膜肺母细胞瘤与囊性肾瘤的关联：可能的遗传关联。

An association of pleuropulmonary blastoma and cystic nephroma: possible genetic association.

作者信息

Bouron-Dal Soglio Dorotheé, Harvey Isabelle, Yazbeck Salam, Rypens Françoise, Oligny Luc L, Fournet Jean-Christophe

机构信息

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

出版信息

Pediatr Dev Pathol. 2006 Jan-Feb;9(1):61-4. doi: 10.2350/08-05-0091.1. Epub 2006 Apr 4.

DOI:10.2350/08-05-0091.1

PMID:16808637

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.

摘要

胸膜肺母细胞瘤与囊性肾瘤的关联是一种罕见情况，英文文献中仅描述了4例同一患者同时存在这两种关联的病例。我们报告了第5例经组织学证实的病例，患者为一名32个月大的男孩。该男孩接受了肺活检，结果显示为胸膜肺母细胞瘤，还进行了肾切除术，结果显示为囊性肾瘤。胸膜肺肿块在活检后化疗后出现显著消退，从而得以进行后续的肿瘤切除术。迄今为止，对于这种罕见情况知之甚少，人们推测这两种肿瘤之间存在遗传联系。