Hodgkin Lymphoma involving the CNS: An AHOD1331, PHL-C1 and PHL-C2 Report from the Children's Oncology Group and the European Network for Pediatric Hodgkin Lymphoma

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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. We describe 45 pediatric patients with 55 extra-axial CNS lesions at the time of 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 in our cohort had a single CNS lesion in the thoracic, lumbar or sacral spine. Two patients (3.6%) had lesions involving the skull. In this 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 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 of these 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. 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 CNS space, and respond similarly to other nodal and extra-nodal disease. Publication History Article published online: 02 December 2025 © 2025. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

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