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卡尼三联征中的胃自主神经(GAN)肿瘤和肾上腺外副神经节瘤。一个共同起源。

Gastric autonomic nerve (GAN) tumor and extra-adrenal paraganglioma in Carney's triad. A common origin.

作者信息

Tortella B J, Matthews J B, Antonioli D A, Dvorak A M, Silen W

出版信息

Ann Surg. 1987 Mar;205(3):221-5. doi: 10.1097/00000658-198703000-00001.

Abstract

Carney's Triad comprises a triad of neoplasms: gastric stromal tumor, extra-adrenal paraganglioma (usually functional), and pulmonary chondroma. At least two of these are needed for the presumptive diagnosis of the Triad. This report presents a patient who had resected a gastric tumor and nonfunctional extra-adrenal paraganglioma. The gastric tumor resembled a gastric leiomyosarcoma by light microscopy, but electron microscopy revealed it to be a gastric autonomic nerve (GAN) tumor. Based on this evidence it appears that both the gastric lesions and the paragangliomata of Carney's Triad are tumors of the autonomic nervous system. Thus, the Triad may be a disorder of the autonomic nervous system rather than a multiple endocrine neoplasia syndrome or multiple hamartoma syndrome.

摘要

卡尼三联征由三种肿瘤组成

胃间质瘤、肾上腺外副神经节瘤(通常有功能)和肺软骨瘤。推定诊断卡尼三联征至少需要其中两种情况。本报告介绍了一名切除胃肿瘤和无功能肾上腺外副神经节瘤的患者。胃肿瘤在光学显微镜下类似胃平滑肌肉瘤,但电子显微镜显示它是一种胃自主神经(GAN)肿瘤。基于这一证据,卡尼三联征的胃病变和副神经节瘤似乎都是自主神经系统的肿瘤。因此,三联征可能是自主神经系统的一种疾病,而非多发性内分泌肿瘤综合征或多发性错构瘤综合征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bb43/1492725/12252f3eb1f0/annsurg00205-0012-a.jpg

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