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罕见的淀粉样变的鼻咽部表现。

A Rare Nasopharyngeal Presentation of Amyloidosis.

机构信息

Head and Neck Oncology Unit, 18524Candiolo Cancer Institute, FPO-IRCCS, Candiolo, Torino, Italy.

Pathology Unit, 18524Candiolo Cancer Institute, FPO - IRCCS, Candiolo, Torino, Italy.

出版信息

Ear Nose Throat J. 2021 Sep;100(8):562-565. doi: 10.1177/0145561320914432. Epub 2020 Mar 30.

Abstract

Amyloidosis is a heterogeneous group of diseases characterized by the extracellular deposition of insoluble proteins whose pathogenesis is not yet fully understood. The deposition of amyloid proteins can be systemic or localized, idiopathic or related to systemic diseases, mostly multiple myeloma or chronic inflammatory diseases. Localized head and neck amyloidosis is a rare entity, mainly involving the larynx. Given the rarity of the disease and the absence of a lasting follow-up protocol, there is no standard treatment defined for localized amyloidosis. We report a rare case of localized nasopharyngeal amyloidosis, treated with complete transoral resection and confirmed by histological examination.

摘要

淀粉样变性是一组异质性疾病,其特征是细胞外不可溶蛋白质的沉积,其发病机制尚不完全清楚。淀粉样蛋白的沉积可以是全身性的或局限性的,特发性的或与系统性疾病相关,主要是多发性骨髓瘤或慢性炎症性疾病。局限性头颈部淀粉样变性是一种罕见的实体,主要累及喉部。由于该疾病的罕见性和缺乏持久的随访方案,局限性淀粉样变性没有定义的标准治疗方法。我们报告了一例罕见的鼻咽部局限性淀粉样变性病例,采用经口完全切除术治疗,并通过组织学检查得到证实。

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