Institut Curie, Department of Radiotherapy, Paris, France.
Hôpital Cochin, Department of Hematology, Paris, France.
Clin Lymphoma Myeloma Leuk. 2022 Nov;22(11):841-846. doi: 10.1016/j.clml.2022.07.003. Epub 2022 Jul 11.
Plasma cell neoplasms are exceptionally rare in the pediatric population; the demographic characteristics and the clinical outcomes of plasma cell neoplasms in this population are currently poorly understood. The aim of this study was to provide a comprehensive analysis of pediatric plasma cell neoplasms, based on the United-States Surveillance, Epidemiology, and End Results (SEER) program registries.
All pediatric patients (aged less than 20 years) diagnosed with a malignant plasma cell neoplasm were retrieved from the SEER Program database (18 registries), collecting patient records between 2000 and 2018. The plasma cell neoplasm type, sex, age at diagnosis, year of diagnosis, race and origin, primary disease site, follow-up duration, and vital status at the last known contact were retrieved and analyzed.
The age-adjusted incidence rate of plasma cell neoplasms for 1,000,000 person-years was 0.06 for the pediatric population (compared with 90.6 for the adult population). The types of pediatric plasma cell neoplasms predominantly consisted of plasmacytomas, with 11 solitary extraosseous plasmacytoma (42.3%) and 7 solitary bone plasmacytoma (26.9%), while plasma cell myelomas represented only a minority of the neoplasms (8 patients; 30.8%). Most plasmacytomas were localized in the head and neck region. Hispanic patients represented 50% of the pediatric plasma cell neoplasm cases (but only 11.1% of adult cases, P < .01). Female-to-Male ratio was 1.36. Five-year overall survival rates were 88.2% (95% confidence interval [95% CI]: 74.2%-100%) for pediatric plasmacytoma and 36.5% (95% CI: 12.4%-100%) for pediatric plasma cell myeloma (P = .013).
This first population-based study of pediatric plasma cell neoplasms underlines the rarity of this entity and demonstrates its unique characteristics, including the significant predominance of plasmacytomas, of female patients, and of patients from hispanic origin, and the poor clinical outcomes of pediatric plasma cell myeloma patients.
浆细胞肿瘤在儿科人群中极为罕见；目前，人们对该人群中浆细胞肿瘤的人口统计学特征和临床结局知之甚少。本研究的目的是基于美国监测、流行病学和最终结果（SEER）计划登记处，对儿科浆细胞肿瘤进行全面分析。
从 SEER 计划数据库（18 个登记处）中检索到所有诊断为恶性浆细胞肿瘤的儿科患者（年龄小于 20 岁），收集 2000 年至 2018 年的患者记录。检索并分析了浆细胞肿瘤类型、性别、诊断时年龄、诊断年份、种族和来源、原发疾病部位、随访时间和最后一次已知接触时的生存状态。
按每 100 万人年计算，儿科浆细胞肿瘤的年龄调整发病率为 0.06（成人发病率为 90.6）。儿科浆细胞肿瘤的类型主要为浆细胞瘤，其中 11 例为孤立性骨外浆细胞瘤（42.3%）和 7 例孤立性骨浆细胞瘤（26.9%），而浆细胞骨髓瘤仅占少数（8 例；30.8%）。大多数浆细胞瘤位于头颈部。西班牙裔患者占儿科浆细胞肿瘤病例的 50%（但仅占成人病例的 11.1%，P<.01）。男女比例为 1.36。儿科浆细胞瘤的 5 年总生存率为 88.2%（95%置信区间[95%CI]：74.2%-100%），而儿科浆细胞骨髓瘤的 5 年总生存率为 36.5%（95%CI：12.4%-100%）（P=.013）。
这是第一项基于人群的儿科浆细胞肿瘤研究，强调了这种疾病的罕见性，并证明了其独特的特征，包括浆细胞瘤的显著优势、女性患者的显著优势和西班牙裔患者的显著优势，以及儿科浆细胞骨髓瘤患者的不良临床结局。
