累及中枢神经系统（CNS）的霍奇金淋巴瘤（HL）极为罕见。有关 CNS HL 患者的表现、管理、治疗和结果的信息仅限于病例报告或小系列研究。我们描述了 45 名在诊断为 HL 时患有 55 个轴外 CNS 病变的儿科患者，这些患者来自儿童肿瘤学组 AHOD1331 和欧洲儿科霍奇金淋巴瘤网络 (EuroNet-PHL) C1 和 C2 试验（NCT02166463、NCT00433459 和 NCT00433459）入组的 4995 名患者。 NCT02684708，clinicaltrials.gov），总发生率为 0.9%。 82.2%的患者在胸椎、腰椎或骶椎有单一中枢神经系统病变。在评估的队列中，HL 并未发生在中枢神经系统实质内。病变从邻近的软组织或骨骼延伸到轴外中枢神经系统间隙，但从未直接穿过硬脑膜渗透到大脑或脊髓。中枢神经系统受累患者的结外病变（E 病变）发生率比之前报道的无中枢神经系统受累患者高出 2 倍。 89.1% 的中枢神经系统病变在两个周期的化疗后表现出完全的代谢反应，并且体积减少 >75%。 13个中枢神经系统病灶（23.6%）接受了照射，没有出现疾病复发的部位。复发发生在涉及中枢神经系统的两个病变部位，这两个病变对化疗都有足够的中期反应。总之，我们提出了诊断时涉及中枢神经系统的最大的系统性 HL 队列，证明这些病变起源于周围组织，延伸到轴外中枢神经系统空间，并且与其他结节和结外疾病的反应类似。版权所有 © 2024 American血液学会。

Hodgkin lymphoma (HL) involving the central nervous system (CNS) is exceedingly rare. Information regarding the presentation, management, treatment and outcome of patients with CNS HL is limited to case reports or small series. We describe 45 pediatric patients with 55 extra-axial CNS lesions at diagnosis with HL from a cohort of 4995 patients enrolled on Children's Oncology Group AHOD1331 and the European Network for Pediatric Hodgkin lymphoma (EuroNet-PHL) C1 and C2 trials (NCT02166463, NCT00433459 and NCT02684708, clinicaltrials.gov), with an overall incidence of 0.9%. 82.2% of patients had a single CNS lesion in the thoracic, lumbar or sacral spine. In the evaluated cohort, HL did not occur within the CNS parenchyma. Lesions extended into the extra-axial CNS space from adjacent soft tissue or bone and never directly infiltrated through the dura into the brain or spinal cord. Patients with CNS involvement had a 2-fold greater incidence of extranodal lesions (E-lesions) than previously reported cohorts without CNS involvement. 89.1% of CNS lesions demonstrated a complete metabolic response and >75% decrease in volume after two cycles of chemotherapy. Thirteen CNS lesions (23.6%) received irradiation, none were sites of disease relapse. Relapse occurred at the site of two lesions involving the CNS, both of which had an adequate interim response to chemotherapy. In summary, we present the largest reported cohort of systemic HL involving the CNS at diagnosis, demonstrating that these lesions originate from surrounding tissues, extend into the extra-axial CNS space, and respond similarly to other nodal and extranodal disease.Copyright © 2024 American Society of Hematology.