Szczepanek Elżbieta, Drozd-Sokołowska Joanna, Sokołowski Jacek, Rzepakowska Anna, Moskwa Arkadiusz, Pachla Jakub, Grzybowski Jakub, Woźnica Katarzyna, Niemczyk Kazimierz, Jamroziak Krzysztof
Department of Otorhinolaryngology, Head and Neck Surgery, Medical University of Warsaw, 02-097 Warsaw, Poland.
Doctoral School in Medical Sciences and Health Sciences, Jagiellonian University Medical College, 31-530 Cracow, Poland.
J Clin Med. 2022 Jul 28;11(15):4390. doi: 10.3390/jcm11154390.
The involvement of the larynx in plasma cell myeloma (PCM) may manifest as solitary extramedullary plasmacytoma of the larynx (sEMP-L) or as infiltration of the larynx during newly diagnosed or relapsed systemic disease with bone marrow involvement (plasma cell myeloma with laryngeal involvement, PCM-L). To increase knowledge about these rare conditions, we performed a retrospective analysis along with a comprehensive literature review of cases of sEMP-L or PCM-L. Six patients (two sEMP-L and four PCM-L) were identified in our tertiary laryngological centre from 2009 to 2021, constituting 0.88% of all malignant laryngeal tumours. The literature search yielded 187 cases, including 152 sEMP-L and 35 sPCM-L. A comparison of baseline characteristics between sEMP-L and PCM-L performed in the combined cohort of cases from literature review and retrospective analysis revealed that patients with sEMP-L were younger (56 vs. 64 years, ≤ 0.001) and presented less commonly with thyroid or cricoid cartilage involvement (2.2% vs. 30.8%, ≤ 0.001). The prognosis of sEMP-L was better than PCM-L (overall survival 86% vs. 55% at 5 years, = 0.002). Analysis of potential factors that could influence progression-free survival (PFS) in the group of sEMP-L revealed that male sex and cartilage involvement negatively affected PFS in univariate analyses, while only cartilage involvement retained statistical significance in multivariate analysis (HR = 19.94, = 0.024). In conclusion, PCM with laryngeal involvement is sporadic. Secondary involvement of the larynx during PCM might be more common than sEMP-L and is associated with worse survival. The involvement of cartilage adversely influences the outcome of sEMP-L.
喉在浆细胞骨髓瘤（PCM）中的累及情况可能表现为喉孤立性髓外浆细胞瘤（sEMP-L），或在新诊断或复发的伴有骨髓受累的系统性疾病期间出现喉浸润（伴有喉受累的浆细胞骨髓瘤，PCM-L）。为了增加对这些罕见情况的了解，我们进行了一项回顾性分析，并对sEMP-L或PCM-L病例进行了全面的文献综述。2009年至2021年期间，我们在三级喉科中心共识别出6例患者（2例sEMP-L和4例PCM-L），占所有恶性喉肿瘤的0.88%。文献检索共得到187例病例，其中包括152例sEMP-L和35例PCM-L。在文献综述和回顾性分析相结合的病例队列中，对sEMP-L和PCM-L的基线特征进行比较后发现，sEMP-L患者更年轻（56岁对64岁，P≤0.001），甲状腺或环状软骨受累的情况也较少见（2.2%对30.8%，P≤0.001）。sEMP-L的预后优于PCM-L（5年总生存率分别为86%和55%，P = 0.002）。对可能影响sEMP-L组无进展生存期（PFS）的潜在因素进行分析发现，在单因素分析中，男性和软骨受累对PFS有负面影响，而在多因素分析中，只有软骨受累具有统计学意义（HR = 19.94，P = 0.024）。总之，伴有喉受累的PCM是散发性的。PCM期间喉的继发性受累可能比sEMP-L更常见，且与较差的生存率相关。软骨受累对sEMP-L的预后有不利影响。
