Department of Pediatric Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Institute of Artificial Intelligence, Hefei Comprehensive National Science Center, Hefei, China.
Childs Nerv Syst. 2023 Aug;39(8):2037-2043. doi: 10.1007/s00381-023-06021-z. Epub 2023 Jul 4.
Primary central nervous system lymphoma (PCNSL) in pediatric patients presents diagnostic and treatment challenges, leading to delays and suboptimal strategies. Moreover, PCNSL in immunocompetent pediatric patients is rarely reported. This retrospective study aimed to describe the demographic and clinical features, as well as outcomes, of pediatric PCNSL cases.
A retrospective review was conducted on 11 immunocompetent pediatric patients diagnosed with PCNSL between January 2012 and April 2020. Data regarding age, gender, initial presenting symptoms, tumor location, and radiological characteristics were collected. Treatment strategies and analyzed prognosis were documented. Survival curves were generated using the Kaplan-Meir method, and data were analyzed using SPSS (version 23.0, IBM Corp.).
The study cohort comprised 11 patients, including 10 males and 1 female. The age at diagnosis ranged from 4 to 15 years, with a median age of 10.6 years. Headache was the most common presenting symptom, observed in 81.8% (9/11) of patients. Tumor locations in the supratentorial and infratentorial regions exhibited a similar occurrence rate. All tumors showed strong contrast enhancement on T1-weighted images. The average survival time for the 11 patients was 44.4 months. Among them, 5 patients died by the last follow-up visit, with a mean survival time of 8.8 months (one patient died in a car accident).
Headache is the predominant manifestation of PCNSL in pediatric patients. PCNSL demonstrates imaging characteristics resembling various intracranial tumors and is associated with a poor prognosis. Therefore, pediatric neurosurgeons should exercise caution in diagnosing and treating intracranial lymphoma.
原发性中枢神经系统淋巴瘤（PCNSL）在儿科患者中存在诊断和治疗方面的挑战，导致诊断延误和治疗策略欠佳。此外，免疫功能正常的儿科患者中的 PCNSL 很少见。本回顾性研究旨在描述儿科 PCNSL 病例的人口统计学和临床特征以及结局。
对 2012 年 1 月至 2020 年 4 月期间诊断为 PCNSL 的 11 例免疫功能正常的儿科患者进行了回顾性分析。收集了年龄、性别、首发症状、肿瘤部位和影像学特征等数据。记录了治疗策略和分析预后。使用 Kaplan-Meier 方法生成生存曲线，并使用 SPSS（版本 23.0，IBM 公司）进行数据分析。
研究队列包括 11 例患者，其中男 10 例，女 1 例。诊断时的年龄为 4 至 15 岁，中位年龄为 10.6 岁。头痛是最常见的首发症状，81.8%（9/11）的患者存在头痛。幕上和幕下肿瘤的发生率相似。所有肿瘤在 T1 加权图像上均表现为明显的对比增强。11 例患者的平均生存时间为 44.4 个月。其中，5 例患者在最后一次随访时死亡，平均生存时间为 8.8 个月（1 例患者死于车祸）。
头痛是儿科 PCNSL 的主要表现。PCNSL 的影像学特征类似于各种颅内肿瘤，预后较差。因此，儿科神经外科医生在诊断和治疗颅内淋巴瘤时应谨慎。
