Wentland Kelly, Shukairy Mohammad K, Picken Maria M, Patadia Monica O
Otolaryngology, Loyola University Medical Center, Maywood, USA.
Pathology, Loyola University Medical Center, Maywood, USA.
Cureus. 2023 May 15;15(5):e39039. doi: 10.7759/cureus.39039. eCollection 2023 May.
Amyloidosis is the process of extracellular deposition of protein fibrils and manifests pathologically as a systemic or localized process. Localized amyloidosis of the head and neck is uncommon, and involvement of the sphenoid sinus is exceedingly rare. We describe a case of localized amyloidosis isolated from the sphenoid sinus. A descriptive literature search was conducted to highlight presentation, management, and outcomes related to this pathology. Our patient was a 65-year-old male who presented to our clinic with nasal congestion and an incidental finding of a large expansile mass within the sphenoid sinuses. The mass was seen to displace the pituitary gland, and thus a multidisciplinary care approach ensued. The mass was removed via a transnasal endoscopic approach. Pathology revealed fibrocollagenous tissue with calcifications that were positive on Congo red staining. The patient underwent further workup to rule out systemic involvement, which was unremarkable. Based on the findings of his workup, he was ultimately diagnosed with localized amyloidosis. A comprehensive review of the literature revealed 25 other reported cases of localized amyloidosis within the sinonasal region, with only one other case of isolated sphenoid sinus disease. Common presenting symptoms are nonspecific and may mimic other, more frequently seen regional pathologies, including nasal obstruction, rhinorrhea, and epistaxis. The treatment for localized disease is surgical resection. While localized amyloidosis within the sinonasal region is rare, it is important to recognize, work up, and treat it appropriately. A multidisciplinary team approach is necessary for appropriate diagnosis and management, and these patients should be followed closely after treatment.
淀粉样变性是蛋白质原纤维在细胞外沉积的过程，在病理上表现为全身性或局限性过程。头颈部局限性淀粉样变性并不常见，蝶窦受累极为罕见。我们描述了一例孤立于蝶窦的局限性淀粉样变性病例。进行了描述性文献检索，以突出与该病理相关的表现、治疗和结果。我们的患者是一名65岁男性，因鼻塞就诊于我们的诊所，偶然发现蝶窦内有一个巨大的膨胀性肿块。该肿块被观察到推移了垂体，因此采取了多学科护理方法。通过经鼻内镜手术切除了肿块。病理显示为有钙化的纤维胶原组织，刚果红染色呈阳性。患者接受了进一步检查以排除全身受累，结果无异常。根据检查结果最终诊断为局限性淀粉样变性。对文献的全面回顾发现，鼻窦区域还有25例其他报道的局限性淀粉样变性病例，仅有另一例孤立的蝶窦疾病。常见的症状不具有特异性，可能类似于其他更常见的局部病变，包括鼻塞、流涕和鼻出血。局限性疾病的治疗方法是手术切除。虽然鼻窦区域的局限性淀粉样变性很少见，但认识、检查并适当治疗它很重要。多学科团队方法对于正确的诊断和管理是必要的，并且这些患者在治疗后应密切随访。
