Brain Tumor Res Treat.  2024 Jan;12(1):63-69. 10.14791/btrt.2023.0041.

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

• Lee H
• Ahn S
• Cha SH
• Cho WH

• KMID: 2552341
• DOI: http://doi.org/10.14791/btrt.2023.0041

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.

Keyword

Hodgkin disease; Brain metastases; Brain neoplasms; Central nervous system neoplasms; Intracranial neoplasms; Lymphoma; B-cell

Figure

• 

  Fig. 1 Pre- and postoperative MRI findings. A: A contrast-enhanced MRI displaying a lesion measuring approximately 4.5 cm, characterized by a well-defined margin, strong enhancement, and invasion into the dura mater and superior sagittal sinus. B: A T2 FLAIR image illustrating diffuse cerebral edema. C and D: Postoperative T1-enhanced MRI and T2 FLAIR images, respectively, depicting a reduced enhancement of the lesion and a decrease in brain edema

• 

  Fig. 2 Intraoperative views. A: Observation of severe adhesion of the dura mater to the tumor. B: Visualization of a fibrotic, whitish tumor. C: A representation of the tumor’s marginal dissection. D: Completion of the subtotal resection.

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  Fig. 3 Histopathological examination (×400 magnification). A: Hematoxylin and eosin-stained tissue section highlighting characteristic mononucleated Reed-Sternberg (Hodgkin) cells (indicated by the arrows). B and C: Immunohistochemical staining reveals positive reactions for CD30 and CD15. D: Immunohistochemical staining for CD20 indicates negative cell reactions.

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  Fig. 4 Histogram depicting the number of intracranial Hodgkin lymphoma (IC-HL) cases reported in literature from 1980 to 2023 across different age groups. Unlike diffuse large B-cell lymphoma, there is no distinct age-related difference.

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