Bangolo Ayrton I, Fwelo Pierre, Trivedi Chinmay, Sagireddy Sowmya, Aljanaahi Hamed, Auda Auda, Mohamed Maryama, Onyeka Sonia, Fisher Miriam, Thapa Jyoti, Tabucanon Erwin J, Georgiev Lyuben, Wishart Annetta, Kumari Shilpee, Erikson Conrad, Bangura Mary, Paddy Orent, Madhukar Rashmi, Gomez Eugenio L, Rathod Joshua, Naria Mansi, Hajal Basel, Awadhalla Mohammad, Siegel David, Parmar Harsh, Biran Noa, Vesole David H, Phull Pooja, Weissman Simcha
Department of Medicine, Palisades Medical Center, North Bergen, NJ 07047, United States.
Department of Epidemiology, Human Genetics, and Environmental Sciences, UTHealth School of Public Health, Houston, TX 77204, United States.
World J Clin Oncol. 2023 Apr 24;14(4):179-189. doi: 10.5306/wjco.v14.i4.179.
Extramedullary multiple myeloma (MM) (EMM) is a rare and aggressive subentity of MM that can be present at diagnosis or develop anytime during the disease course. There is a paucity of data on the clinical characteristics and overall epidemiology of EMM. Furthermore, there is a scarcity of data on how the interaction of age and gender influences the survival of EMM.
To evaluate the clinical characteristics of patients with EMM over the past 2 decades and to identify epidemiologic characteristics that may impact overall prognosis.
A total of 858 patients diagnosed with EMM, between 2000 and 2017, were ultimately enrolled in our study by retrieving the Surveillance, Epidemiology, and End Results database. We analyzed demographics, clinical characteristics, and overall mortality (OM) as well as cancer-specific mortality (CSM) of EMM. Variables with a value < 0.1 in the univariate Cox regression were incorporated into the multivariate Cox model to determine the independent prognostic factors, with a hazard ratio (HR) of greater than 1 representing adverse prognostic factors.
From a sample of 858 EMM, the male gender (63.25%), age range 60-79 years (51.05%), and non-Hispanic whites (66.78%) were the most represented. Central Nervous System and the vertebral column was the most affected site (33.10%). Crude analysis revealed higher OM in the age group 80+ [HR = 6.951, 95% confidence interval (95%CI): 3.299-14.647, = 0], Non-Hispanic Black population (HR = 1.339, 95%CI: 1.02-1.759, = 0.036), Bones not otherwise specified (NOS) (HR = 1.74, 95%CI: 1.043-2.902, = 0.034), and widowed individuals (HR = 2.107, 95%CI: 1.511-2.938, = 0). Skin involvement (HR = 0.241, 95%CI: 0.06-0.974, = 0.046) and a yearly income of $75000+ (HR = 0.259, 95%CI: 0.125-0.538, = 0) had the lowest OM in the crude analysis. Crude analysis revealed higher CSM in the age group 80+, Non-Hispanic Black, Bones NOS, and widowed. Multivariate cox proportional hazard regression analyses only revealed higher OM in the age group 80+ (HR = 9.792, 95%CI: 4.403-21.774, = 0) and widowed individuals (HR = 1.609, 95%CI: 1.101-2.35, = 0.014). Multivariate cox proportional hazard regression analyses of CSM also revealed higher mortality of the same groups. Eyes, mouth, and ENT involvement had the lowest CSM in the multivariate analysis. There was no interaction between age and gender in the adjusted analysis for OM and CSM.
EMM is a rare entity. To our knowledge, there is a scarcity of data on the clinical characteristics and prognosis factors of patients with extramedullary multiple myeloma. In this retrospective cohort, using a United States-based population, we found that age, marital status, and tumor site were independent prognostic factors. Furthermore, we found that age and gender did not interact to influence the mortality of patients with EMM.
髓外多发性骨髓瘤（EMM）是一种罕见且侵袭性强的骨髓瘤亚型，可在诊断时出现，也可在疾病过程中的任何时间发生。关于EMM的临床特征和总体流行病学的数据很少。此外，关于年龄和性别相互作用如何影响EMM患者生存的数据也很匮乏。
评估过去20年中EMM患者的临床特征，并确定可能影响总体预后的流行病学特征。
通过检索监测、流行病学和最终结果数据库，最终纳入了2000年至2017年间诊断为EMM的858例患者。我们分析了EMM的人口统计学、临床特征、总体死亡率（OM）以及癌症特异性死亡率（CSM）。单因素Cox回归中P值<0.1的变量被纳入多因素Cox模型，以确定独立的预后因素，风险比（HR）大于1表示不良预后因素。
在858例EMM样本中，男性（63.25%）、年龄范围60 - 79岁（51.05%）和非西班牙裔白人（66.78%）占比最高。中枢神经系统和脊柱是最常受累的部位（33.10%）。粗分析显示，80岁以上年龄组的OM较高[HR = 6.951，95%置信区间（95%CI）：3.299 - 14.647，P = 0]、非西班牙裔黑人（HR = 1.339，95%CI：1.02 - 1.759，P = 0.036）、未另行指定的骨骼（NOS）（HR = 1.74，95%CI：1.043 - 2.902，P = 0.034）以及丧偶个体（HR = 2.107，95%CI：1.511 - 2.938，P = 0）。皮肤受累（HR = 0.241，95%CI：0.06 - 0.974，P = 0.046）和年收入75000美元以上（HR = 0.259，95%CI：0.125 - 0.538，P = 0）在粗分析中的OM最低。粗分析显示，80岁以上年龄组、非西班牙裔黑人、骨骼NOS和丧偶个体的CSM较高。多因素Cox比例风险回归分析仅显示80岁以上年龄组的OM较高（HR = 9.792，95%CI：4.403 - 21.774，P = 0）和丧偶个体（HR = 1.609，95%CI：1.101 - 2.35，P = 0.014）。CSM的多因素Cox比例风险回归分析也显示相同组的死亡率较高。在多因素分析中，眼睛、口腔和耳鼻喉受累的CSM最低。在OM和CSM的校正分析中，年龄和性别之间没有相互作用。
EMM是一种罕见的疾病。据我们所知，关于髓外多发性骨髓瘤患者的临床特征和预后因素的数据很少。在这个基于美国人群的回顾性队列研究中，我们发现年龄、婚姻状况和肿瘤部位是独立的预后因素。此外，我们发现年龄和性别不会相互作用来影响EMM患者的死亡率。
