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多发性黏膜神经纤维瘤、嗜铬细胞瘤和甲状腺髓样癌综合征。病例报告。

Syndrome of multiple mucosal neurofibromas, pheochromocytoma and medullary thryoid carcinoma. Report of a case.

作者信息

Anneroth G, Heimdahl A

出版信息

Int J Oral Surg. 1978 Apr;7(2):126-31. doi: 10.1016/s0300-9785(78)80059-3.

Abstract

The present article reports a case of multiple endocrine neoplasia (MEN) syndrome, type IIb, including the following components: pheochromocytoma, medullary thyroid carcinoma, hyperplastic corneal nerves and multiple mucosal oral and ocular neurofibromas. The patients, a 35-year-old male also exhibited a series of other pertinent findings, i.e. thickened eyelids, marfanoid habitus and widened mandibular canal and mental foramina. In the discussion the clinical and histopathologic characteristics of the present case are compared with similar findings in other previously published cases. The multiple oral mucosal neurofibromas are described clinically and histopathologically and are stated as a pathognomonic early sign of the syndrome.

摘要

本文报道了一例IIb型多发性内分泌腺瘤(MEN)综合征病例,包括以下组成部分:嗜铬细胞瘤、甲状腺髓样癌、角膜神经增生以及口腔和眼部多发性黏膜神经纤维瘤。该患者为一名35岁男性,还表现出一系列其他相关体征,即眼睑增厚、类马凡氏体型以及下颌管和颏孔增宽。在讨论中,将本病例的临床和组织病理学特征与其他先前发表病例的类似发现进行了比较。对多发性口腔黏膜神经纤维瘤进行了临床和组织病理学描述,并指出其为该综合征的特征性早期体征。

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