伴有先天性囊性腺瘤样畸形的胸膜肺母细胞瘤综合征表现。病例报告。

Syndromic presentation of a pleuropulmonary blastoma associated with congenital cystic adenomatoid malformation. A case report.

作者信息

Nur Samina, Badr Riem, Sandoval Claudio, Brudniki Adele, Yeh Albert

机构信息

Department of Pathology, New York Medical College, Westchester Medical Center, Valhalla, NY 10595, USA.

出版信息

J Pediatr Surg. 2007 Oct;42(10):1772-5. doi: 10.1016/j.jpedsurg.2007.06.022.

DOI:10.1016/j.jpedsurg.2007.06.022

PMID:17923214

Abstract

Pleuropulmonary blastoma (PPB) is a rare malignant mesenchymal pediatric tumor with a well-recognized association with congenital cystic adenomatoid malformation (CCAM). Recently, it has been described in a patient with CCAM, multiple jejunal polyps, and cystic nephroma. We describe a similar case of a unique presentation of PPB, arising in association with CCAM and with a history of intussception caused by multiple small bowel polyps.

摘要

胸膜肺母细胞瘤（PPB）是一种罕见的小儿恶性间充质肿瘤，与先天性囊性腺瘤样畸形（CCAM）有明确关联。最近，在一名患有CCAM、多发空肠息肉和囊性肾瘤的患者中对其进行了描述。我们描述了一例类似的PPB独特表现病例，该病例与CCAM相关，并伴有由多个小肠息肉引起的肠套叠病史。