Nagasaka T, Lai R, Kuno K, Nakashima T, Nakashima N
Division of Pathology, Clinical Laboratory, Nagoya University Hospital, Nagoya University, School of Medicine, Nagoya, Japan.
Hum Pathol. 2001 Jan;32(1):132-4. doi: 10.1053/hupa.2001.20896.
Extramedullary plasmacytoma of the larynx and localized laryngeal amyloidosis are 2 uncommon disease entities that are exceedingly rare in children. We report a case of a 12-year-old girl presenting with progressive hoarseness who was subsequently found to have extramedullary plasmacytoma coexisting with localized amyloidosis involving the larynx. Results from the immunohistochemical and molecular studies showed that the tumor cells of the plasmacytoma were monoclonal (kappa-restricted), strongly supporting their neoplastic nature. The biochemical nature of the amyloid deposits was also shown to be of kappa immunoglobulin light chain, suggesting the pathogenetic relationship between the plasmacytoma and amyloid deposition in the larynx of this patient. There was no other evidence of malignancy or amyloidosis elsewhere. On a follow-up period of 4 years, this patient was well and asymptomatic. We believe that this represents the first case in the literature showing the coexistence of extramedullary plasmacytoma and localized amyloidosis of the larynx in children. This case also supports the concept that localized laryngeal amyloidosis may be a manifestation of low-grade B-cell neoplasms.
喉髓外浆细胞瘤和局限性喉淀粉样变性是两种罕见的疾病实体,在儿童中极为罕见。我们报告一例12岁女孩,表现为进行性声音嘶哑,随后发现患有髓外浆细胞瘤并伴有累及喉部的局限性淀粉样变性。免疫组织化学和分子研究结果显示,浆细胞瘤的肿瘤细胞是单克隆的(κ链受限),有力地支持了它们的肿瘤性质。淀粉样沉积物的生化性质也显示为κ免疫球蛋白轻链,提示该患者喉内浆细胞瘤与淀粉样沉积之间的发病机制关系。其他部位没有恶性肿瘤或淀粉样变性的其他证据。在4年的随访期内,该患者情况良好且无症状。我们认为这是文献中首例显示儿童喉髓外浆细胞瘤与局限性淀粉样变性共存的病例。该病例也支持局限性喉淀粉样变性可能是低度B细胞肿瘤表现的观点。