肺多发性软骨瘤性错构瘤：一例报告并结合文献复习，特别提及卡尼综合征
Multiple chondromatous hamartomas of the lung: a case report and review of the literature with special reference to Carney syndrome.
作者信息
Kiryu T, Kawaguchi S, Matsui E, Hoshi H, Kokubo M, Shimokawa K
机构信息
Department of Radiology, Gifu University School of Medicine, Gifu City, Japan.
出版信息
Cancer. 1999 Jun 15;85(12):2557-61. doi: 10.1002/(sici)1097-0142(19990615)85:12<2557::aid-cncr10>3.0.co;2-2.
BACKGROUND
Multiple chondromatous hamartomas of the lung, which are very rare, are a feature of Carney syndrome. The relation between the two entities is not clear.
METHODS
A patient with multiple chondromatous hamartomas of the lung is described in this article. The literature was reviewed with special reference to the relation between multiple chondromatous hamartomas of the lung and Carney syndrome as well as the triad of gastric epithelioid leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma.
RESULTS
A total of 15 cases of multiple chondromatous hamartomas of the lung have been published worldwide. Two cases exhibited two other features of Carney syndrome, namely, gastric leiomyogenic neoplasms and extra-adrenal paragangliomas, and three other cases demonstrated only gastric leiomyomatous neoplasms. These five patients were all young females.
CONCLUSIONS
Some patients with multiple chondromatous hamartomas of the lung have a history of Carney syndrome. Patients with multiple chondromatous hamartomas require further examination of other sites, particularly the stomach and nervous system.
背景
肺部多发性软骨瘤样错构瘤极为罕见，是卡尼综合征的一个特征。这两种病症之间的关系尚不清楚。
方法
本文描述了一名患有肺部多发性软骨瘤样错构瘤的患者。回顾了相关文献，特别提及肺部多发性软骨瘤样错构瘤与卡尼综合征之间的关系，以及胃上皮样平滑肌肉瘤、功能性肾上腺外副神经节瘤和肺软骨瘤三联征。
结果
全球共发表了15例肺部多发性软骨瘤样错构瘤病例。其中2例表现出卡尼综合征的另外两个特征，即胃平滑肌瘤性肿瘤和肾上腺外副神经节瘤，另外3例仅表现出胃平滑肌瘤性肿瘤。这5名患者均为年轻女性。
结论
一些患有肺部多发性软骨瘤样错构瘤的患者有卡尼综合征病史。患有肺部多发性软骨瘤样错构瘤的患者需要进一步检查其他部位，特别是胃和神经系统。
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