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卡尼综合征的评估与管理:一个实例

Evaluation and management of Carney's complex: an illustrative case.

作者信息

Wallace T M, Levin H S, Ratliff N B, Hobbs R E

机构信息

Department of Pathology, Cleveland Clinic Foundation, Ohio 44195.

出版信息

Cleve Clin J Med. 1991 May-Jun;58(3):248-50, 255-6. doi: 10.3949/ccjm.58.3.248.

Abstract

Carney's complex is characterized by cardiac and cutaneous myxomas, mammary myxoid fibroadenomas, spotty mucocutaneous pigmentation, primary pigmented adrenocortical disease, large-cell calcifying Sertoli cell tumors of the testis, growth hormone-secreting pituitary adenomas, and psammomatous melanotic schwannomas. We present an illustrative case of Carney's complex in a 26-year-old woman who at age 20 underwent bilateral adrenalectomy for Cushing's syndrome. Six years later, she suffered multiple central nervous system embolic episodes secondary to three cardiac myxomas, which led to her death. Appropriate evaluation and management of patients with Carney's complex is outlined.

摘要

卡尼综合征的特征包括心脏和皮肤黏液瘤、乳腺黏液样纤维腺瘤、散在性黏膜皮肤色素沉着、原发性色素沉着肾上腺皮质疾病、睾丸大细胞钙化性支持细胞瘤、分泌生长激素的垂体腺瘤以及砂粒样黑色素性神经鞘瘤。我们报告一例26岁女性卡尼综合征的典型病例,该患者20岁时因库欣综合征接受双侧肾上腺切除术。六年后,她因三个心脏黏液瘤继发多次中枢神经系统栓塞发作,最终导致死亡。本文概述了卡尼综合征患者的适当评估和管理。

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