Department of Pathology, Immunology and Laboratory Medicine, University of Florida College of Medicine, Gainesville, FL, U.S.A.
Department of Otolaryngology-Head and Neck Surgery, Xiangya School of Medicine, Central South University, Changsha, P.R. China.
In Vivo. 2020 Jan-Feb;34(1):255-260. doi: 10.21873/invivo.11768.
Laryngeal stenosis is challenging for treatment due to uncertain etiology. Primary laryngeal lymphoma as the initial clinical manifestation of laryngeal stenosis has been rarely reported. Primary diffuse large B-cell lymphoma as an underlying etiology has not been reported.
A 79-year-old male presented with dyspnea, stridor and dysphonia of 6 months' duration. Computed tomography scans and flexible laryngoscopic examination revealed vocal cord mobility with bilaterally limited abduction and a subglottic stenosis up to 50%. The laryngeal mucosa was smooth. Laryngeal biopsy showed atypical lymphoid infiltrates, predominantly large sized B-cells, in the submucosa with crush/cauterized artifacts. The tumor cells were positive for B-lymphocyte antigen CD20, paired-box 5 (PAX5), B-cell lymphoma 2 (BCL2), BCL6 and multiple myeloma oncogene 1 (MUM1). They were negative for CD10, CD30, cyclin D1 (CCND1), SRY-box 11 (SOX11), activin-receptor like kinase 1 (ALK1), CD138 and c-MYC, and negative for kappa/lambda light chain and Epstein-Barr virus-encoded small RNA by in situ hybridization. The pathologic diagnosis was diffuse large B-cell lymphoma. Fluorescent in situ hybridization (FISH) for MYC was negative. Next-generation sequencing using a 175-gene panel was performed and no pathologic mutations were identified. No lymphadenopathy elsewhere was identified. The patient was treated with chemotherapy and was doing well at the 5-month follow-up.
To the best of our knowledge, this is the first documented case of primary laryngeal diffuse large B-cell lymphoma presenting as increasing laryngeal stenosis. The rarity, diagnosis and treatment of this entity are discussed.
由于病因不确定,喉狭窄的治疗具有挑战性。原发性喉淋巴瘤作为喉狭窄的初始临床表现很少见报道。原发性弥漫性大 B 细胞淋巴瘤作为潜在病因尚未报道。
一名 79 岁男性,以呼吸困难、喘鸣和声音嘶哑 6 个月就诊。计算机断层扫描和纤维喉镜检查显示声带活动度正常,但双侧外展受限,声门下狭窄达 50%。喉黏膜光滑。喉活检显示黏膜下有典型的淋巴样浸润,主要为大尺寸 B 细胞,伴有挤压/烧灼伪影。肿瘤细胞表达 B 淋巴细胞抗原 CD20、配对盒 5(PAX5)、B 细胞淋巴瘤 2(BCL2)、BCL6 和多发性骨髓瘤癌基因 1(MUM1)。它们不表达 CD10、CD30、细胞周期蛋白 D1(CCND1)、SRY 盒 11(SOX11)、激活素受体样激酶 1(ALK1)、CD138 和 c-MYC,并且原位杂交检测到 kappa/ lambda 轻链和 Epstein-Barr 病毒编码的小 RNA 均为阴性。病理诊断为弥漫性大 B 细胞淋巴瘤。荧光原位杂交(FISH)检测 MYC 为阴性。随后进行了 175 个基因panel 的下一代测序,未发现病理突变。未发现其他部位的淋巴结病。患者接受化疗,随访 5 个月时情况良好。
据我们所知,这是首例原发性喉弥漫性大 B 细胞淋巴瘤表现为进行性喉狭窄的病例。讨论了该实体的罕见性、诊断和治疗。