Takakura Hiromasa, Tachino Hirohiko, Takii Kouji, Imura Johji, Shojaku Hideo
Department of Otorhinolaryngology, Head and Neck Surgery, Faculty of Medicine, Academic Assembly, University of Toyama, Toyama, Japan.
Department of Diagnostic Pathology, Faculty of Medicine, Academic Assembly, University of Toyama, Toyama, Japan.
Front Surg. 2021 Nov 5;8:774469. doi: 10.3389/fsurg.2021.774469. eCollection 2021.
Amyloidosis is a disorder of protein folding in which various proteins automatically aggregate into a highly abnormal fibrillar conformation. Amyloidosis is classified into systemic and localized forms depending on whether the abnormal proteins deposited in several different organs or only a single organ. In localized amyloidosis of the head and neck regions, laryngeal amyloidosis is common; however, localized amyloidosis of the nose is extremely rare. We herein report a case of localized amyloidosis of the nose and review the relevant literature on localized sinonasal amyloidosis. A 41-year-old man presented with a history of severe nasal obstruction, which had persisted for two decades. Nasal endoscopy and imaging studies showed extensive thickening of the bilateral nasal mucosa and diffuse submucosal deposition of calcification. After histopathological and systemic examinations, he was diagnosed with localized amyloidosis of the nasal mucosa. Septoplasty and bilateral inferior turbinoplasty, which consisted of mucosal resection using an ultrasonic bone curette, was performed and his symptoms markedly improved. Localized sinonasal amyloidosis has a good prognosis and surgical resection should be selected as a first-line treatment; however, clinicians should recognize the high probability of recurrence.
淀粉样变性是一种蛋白质折叠紊乱疾病,其中各种蛋白质会自动聚集成高度异常的纤维状构象。淀粉样变性根据异常蛋白质是沉积在几个不同器官还是仅沉积在单个器官而分为系统性和局限性两种形式。在头颈部区域的局限性淀粉样变性中,喉淀粉样变性较为常见;然而,鼻局限性淀粉样变性极为罕见。我们在此报告一例鼻局限性淀粉样变性病例,并回顾有关局限性鼻鼻窦淀粉样变性的相关文献。一名41岁男性有严重鼻塞病史,持续了二十年。鼻内镜检查和影像学研究显示双侧鼻黏膜广泛增厚以及弥漫性黏膜下钙化沉积。经过组织病理学和全身检查后,他被诊断为鼻黏膜局限性淀粉样变性。实施了鼻中隔成形术和双侧下鼻甲成形术,包括使用超声骨刮匙进行黏膜切除,其症状明显改善。局限性鼻鼻窦淀粉样变性预后良好,手术切除应作为一线治疗方法;然而,临床医生应认识到复发的高可能性。