Musat Gabriela, Evsei Anca, Calina Daniela, Docea Anca Oana, Doukas Sotirios G, Vageli Dimitra P, Nepka Charitini, Spandidos Demetrios A, Mitroi Mihaela
Department of Otorhinolaryngology, University of Medicine and Pharmacy 'Carol Davila', 050474 Bucharest, Romania.
Laboratory of Anatomy-Histology, 'Saint Mary' Clinic Hospital, 011172 Bucharest, Romania.
Mol Clin Oncol. 2020 Mar;12(3):258-262. doi: 10.3892/mco.2020.1972. Epub 2020 Jan 9.
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.
局限性淀粉样变性是一种罕见疾病,其特征为错误折叠的蛋白质在组织中沉积,而无其他全身表现。与全身性淀粉样变性(舌部受累很常见)相比,迄今为止仅报道了少数舌部局限性淀粉样变性病例。本研究报告了一例罕见的舌部局限性淀粉样变性(淀粉样瘤)病例,并总结了局限性淀粉样变性的已知文献。本研究描述了一名36岁女性患者,其表现为舌根肿胀。淀粉样瘤的诊断基于体格检查结果、头颈部MRI检查结果以及偏振光下刚果红染色的组织病理学检查结果。组织病理学诊断如下:局限性λ轻链淀粉样变性。耳鼻喉科和血液学/肿瘤学部门进行了全面的体格检查,未发现全身疾病迹象。还进行了一系列血液学和影像学检查,以证实没有全身受累迹象。患者拒绝手术切除,2年随访未发现肿瘤大小有任何变化。尽管局限性淀粉样变性的病因尚不清楚,但组织浆细胞对环境抗原的长期反应可能是肿瘤形成过程起始的一个致病因素。