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一名年轻女性的钙化性肺肿瘤和小细胞贫血:卡尼三联征的部分表现

A calcified lung tumour and microcytic anaemia in a young woman: partial expression of the Carney triad.

作者信息

Alberto V O, Kelleher D, Denholm R B, Nutt M, Carney J A

机构信息

West Wales General Hospital, Department of Surgery, Carmarthen, UK.

出版信息

Surgeon. 2008 Aug;6(4):249-51. doi: 10.1016/s1479-666x(08)80036-4.

Abstract

The Carney triad is the non-familial addociation of gastric stromal tumours (GISTs), pulmonar chondromas and extra-adrenal paragangliomas. Fewer than 100 cases of the disorder have been reported since its description in 1977. The condition has a predeliction for young women. Most patients exhibit only two of the three components. The tumours tend to be multifocal in the affected organ or system. Herein, we describe the case of a 27-year-old woman with multiple gastric GISTs and a pulmonary chondroma, partial expression of the Carney triad. It is important to be aware of the Carney triad when one of its constituent tumours is found, particularly if the patient is a young woman, so that a search can be made for and surveillance instituted for the other components. Treatment for the gastric tumours (sarcomas) and the paragangliomas (potentially malignant) is surgical. The lung chondromas are benign neoplasms and ordinarily not symptomatic. If a diagnosis of the tumour can be established by biopsy, surgical resection may not be necessary.

摘要

卡尼三联征是胃间质瘤(GISTs)、肺软骨瘤和肾上腺外副神经节瘤的非家族性组合。自1977年首次描述以来,报道的该疾病病例少于100例。这种疾病好发于年轻女性。大多数患者仅表现出三联征中的两个组成部分。肿瘤在受影响的器官或系统中往往呈多灶性。在此,我们报告一例27岁女性,患有多发胃间质瘤和肺软骨瘤,部分表现为卡尼三联征。当发现卡尼三联征的其中一种构成肿瘤时,尤其是患者为年轻女性时,认识到卡尼三联征很重要,以便对其他组成部分进行排查和监测。胃肿瘤(肉瘤)和副神经节瘤(有潜在恶性)的治疗方法是手术。肺软骨瘤是良性肿瘤,通常无症状。如果通过活检能够确诊肿瘤,可能无需手术切除。

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