肺淋巴管肌瘤病和结节性硬化症相关的细支气管肺泡癌：病例报告。

Broncheoalveolar carcinoma associated with pulmonary lymphangioleiomyomatosis and Tuberous Sclerosis Complex: case report.

机构信息

Department of Radiology, Instituto Portugues de Oncologia Francisco Gentil, Porto, Portugal.

出版信息

Clin Imaging. 2011 May-Jun;35(3):225-7. doi: 10.1016/j.clinimag.2010.04.003.

DOI:10.1016/j.clinimag.2010.04.003

Abstract

Lymphangioleiomyomatosis (LAM) is a rare disorder that predominantly affects the lung parenchyma of young women and it's characterized by pulmonary cyst. Tuberous sclerosis complex (TSC) is a rare genetic disorder presenting with hamartomas and neurologic symptoms. The two renal pathologies most commonly seen in TSC are angiomyolipomas and cysts; less commonly, TSC co-exist with polycystic kidney disease. In this report is described an uncommon case of a patient with broncheoalveolar carcinoma, pulmonary LAM and TSC with polycystic kidney disease.

摘要

淋巴管平滑肌瘤病（LAM）是一种罕见疾病，主要影响年轻女性的肺实质，其特征是肺部囊肿。结节性硬化症（TSC）是一种罕见的遗传疾病，表现为错构瘤和神经症状。TSC 中最常见的两种肾脏病变是血管平滑肌脂肪瘤和囊肿；较少见的是，TSC 与多囊肾病并存。本报告描述了一例罕见病例，患者患有支气管肺泡癌、肺 LAM 和 TSC 合并多囊肾病。