局部淀粉样变是一种罕见的疾病,其特征是错误折叠蛋白在组织中沉积,没有其他系统性表现。迄今仅报道了少数舌的局部淀粉样变性病例,与系统性淀粉样变性相反,在舌头上的定位是常见的。这项研究提出了一种罕见的舌头局部淀粉样变性(淀粉样变性)病例,并提供了已知的局部淀粉样变性文献的总结。这项研究描述了一名36岁女性舌根肿胀的情况。根据体格检查的结果诊断为淀粉样瘤,头颈部MRI表现及偏振光下刚果红染色的组织病理学检查。组织病理学诊断如下:局部λ轻链淀粉样变性。耳鼻喉科和血液/肿瘤科进行了彻底的体检,没有发现全身性疾病的迹象.还进行了一系列血液学和成像测试以验证没有全身受累的迹象。患者拒绝手术切除,2年的随访未发现肿瘤尺寸有任何变化。尽管局限性淀粉样变性的病因尚不清楚,组织浆细胞对环境抗原的长时间反应可能是肿瘤形成过程开始的原因。
Localized amyloidosis is a rare condition characterized by the deposition of misfolding protein in a tissue, without other systemic manifestations. Only a small number of cases of localized amyloidosis of the tongue have been reported to date, in contrast to systemic amyloidosis, in which localization on the tongue is common. This study presents a rare
case of localized amyloidosis of the tongue (amyloidoma) and provides a summary of the known literature of localized amyloidosis. This study describes the
case of a 36-year-old female who presented with a swelling of the tongue base. The diagnosis of amyloidoma was made based on the findings of the physical examination, head and neck MRI findings and the histopathological examination with Congo red stain under polarized light. The histopathological diagnosis was as follows: Localized lambda light-chain amyloidosis. A thorough physical examination was performed by the ENT and Hematology/Oncology departments, without revealing signs of systemic disease. A series of hematological and imaging tests were also performed to verify that there was no sign of systemic involvement. The patient declined surgical excision and the 2-year follow-up did not reveal any changes in tumor dimension. Although the etiology of localized amyloidosis is yet not clear, the prolonged reaction of tissue plasma cells to environmental antigens may be a causative factor for the initiation of the neoplastic process.