Park Song I, Ryu Gwanghui
Department of Otorhinolaryngology-Head and Neck Surgery, Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Republic of Korea.
Department of Otorhinolaryngology-Head and Neck Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.
J Rhinol. 2023 Nov;30(3):177-180. doi: 10.18787/jr.2023.00043. Epub 2023 Nov 27.
Amyloidosis is a systemic disease characterized by the accumulation of amyloid protein in multiple organs. Amyloidoma, in contrast, is an uncommon localized form of amyloidosis that presents as a single mass or tumor-like lesion. Primary amyloidoma in the central nervous system is rare, and only a few cases have been reported. Notably, the Gasserian ganglion is the most frequently affected site of amyloidoma in the central nervous system, and progressive trigeminal neuropathy is a characteristic finding. Among these cases, the bilateral occurrence of amyloidoma is exceedingly rare. In this report, we present the case of a 51-year-old woman diagnosed with bilateral trigeminal amyloidoma, confirmed by an endoscopic biopsy via the transsphenoidal approach.
淀粉样变性是一种全身性疾病，其特征是淀粉样蛋白在多个器官中积聚。相比之下，淀粉样瘤是淀粉样变性的一种罕见的局限性形式，表现为单个肿块或肿瘤样病变。中枢神经系统原发性淀粉样瘤罕见，仅有少数病例报道。值得注意的是，半月神经节是中枢神经系统淀粉样瘤最常受累的部位，进行性三叉神经病变是其特征性表现。在这些病例中，双侧淀粉样瘤极为罕见。在本报告中，我们介绍了一例51岁女性被诊断为双侧三叉神经淀粉样瘤的病例，该病例经经蝶窦入路内镜活检得以确诊。
