Department of Otolaryngology-Head and Neck Surgery, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Ear Nose Throat J. 2021 Nov;100(9):NP397-NP401. doi: 10.1177/0145561320922719. Epub 2020 May 4.
We present a case of primary systemic amyloidosis diagnosed by endoscopic sinus surgery. A 75-year-old woman had blurred vision in her left eye; computed tomography and magnetic resonance imaging showed shadows of the bilateral paranasal sinuses. Endoscopic sinus surgery was performed, and amyloidosis was diagnosed by histopathology. She had previously been diagnosed with amyloidosis of the stomach, and therefore, she was diagnosed with primary systemic amyloidosis. A systemic workup for additional amyloid deposits revealed no evidence of other diseases. The patient remained under follow-up without further treatment, as no further amyloid deposition or progression of the lesions was seen. Amyloidosis is a rare condition characterized by the deposition of abnormal protein filaments in the extracellular tissue. Generally, systemic amyloidosis does not involve the head and neck region, and the presence of amyloid in the nasal and paranasal sinus mucosa is more likely to be indicative of a localized process. However, in our patient, the lesions were located in both the sinonasal tract and the stomach, indicating systemic amyloidosis. To our knowledge, there have been no previous reports of systemic amyloidosis involving the sinonasal tract, and therefore, we consider this case to be extremely rare.
我们报告了一例经鼻内镜鼻窦手术诊断的原发性系统性淀粉样变病例。一名 75 岁女性左眼视力模糊;计算机断层扫描和磁共振成像显示双侧鼻窦阴影。行鼻内镜鼻窦手术,组织病理学诊断为淀粉样变性。患者既往曾被诊断为胃淀粉样变性,因此诊断为原发性系统性淀粉样变性。全身检查未发现其他淀粉样物质沉积的证据。由于未发现其他疾病,患者未进一步治疗,且未发现进一步的淀粉样物质沉积或病变进展。淀粉样变性是一种罕见疾病,其特征是异常蛋白丝在细胞外组织中沉积。一般来说,系统性淀粉样变性不涉及头颈部,而鼻和鼻窦黏膜中的淀粉样物质更可能提示局部病变。然而,在我们的患者中,病变位于鼻-鼻窦道和胃,提示系统性淀粉样变性。据我们所知,以前没有报告过涉及鼻-鼻窦道的系统性淀粉样变性,因此我们认为这种情况极为罕见。