Intracranial Involvement of Systemic Hodgkin Lymphoma: A Case Report and Literature Review.

Abstract

A 27-year-old male patient, previously diagnosed with Hodgkin lymphoma (HL), presented with gait disturbance. Brain MRI showed a 4.5 cm mass lesion in the right occipital lobe, suggesting either intracranial involvement of HL or a potential meningioma. Despite high-dose methotrexate and steroid treatment, the patient's symptoms persisted, and imaging showed an enlarging mass, leading to surgical intervention. Histopathological examination confirmed central nervous system (CNS) involvement of HL. Postoperatively, the patient underwent whole-brain radiotherapy and demonstrated marked clinical improvement. Our literature review from 1980 to 2023 identified only 46 cases of intracranial HL (IC-HL), underscoring its rarity. Lymphomas represent 2.2% of brain tumors, with 90%-95% being diffuse large B-cell lymphoma (DLBCL). In contrast, the incidence of CNS-HL patients is a mere 0.02%. Notably, IC-HL and intracranial DLBCL have differences in their typical locations and treatment strategies. Unlike DLBCL, which predominantly appears in the supratentorial region (87%), IC-HL is found there in 61.5% of cases. Additionally, 33.3% of IC-HL cases occur in the cerebellum, with 43.5% associated with posterior circulation regions. Furthermore, while biopsy followed by chemotherapy induction is a common strategy for DLBCL, 81.8% of IC-HL cases underwent surgical resection, and only 18.1% had a biopsy alone. The distinct characteristics of IC-HL tumors, including their larger size, attachment to the dura, and fibrotic nature with clear boundaries, might account for the preference for surgical intervention. The unique features of IC-HL compared to DLBCL highlight the need for distinct considerations in diagnosis and management.