Intracranial Presentation of Systemic Hodgkin's Disease

Abstract

Intracranial involvement by Hodgkin's disease is rare. We report a patient with Hodgkin's disease who had intracranial disease at presentation. We also review the literature pertaining to intracranial Hodgkin's disease. Using the key words "Hodgkin's disease" and "central nervous system (CNS) disease", we searched the Pubmed and Cancerlit databases. References were systematically reviewed and data regarding the following variables was extracted: age, gender, signs and symptoms at presentation, histology of Hodgkin's disease, cerebrospinal fluid analysis, stage and treatment. Only 36 cases of intracranial Hodgkin's disease were identified in the literature. Intracranial Hodgkin's disease at presentation is even more uncommon with only 8 reported cases. Most cases of intracranial involvement by Hodgkin's disease occur at the time of relapse. The most common presenting feature of intracranial Hodgkin's disease is a cranial nerve palsy with brain parenchyma being the most common intracranial site of involvement. Mixed cellularity histology is the most frequent subtype of Hodgkin's disease among these patients and the median survival following intracranial presentation is 46 months. Treatment has varied extensively but includes whole brain radiation with or without combination chemotherapy. Our literature review suggests that the prognosis is not dismal with appropriate treatment.

CNS Hodgkin lymphoma

“…2,9 In most reported cases, CNS involvement occurred in the setting of relapsed systemic disease with up to 61% of cases having extraneural evidence of HL synchronous with CNS disease. 1,2,9 However, 5 patients in our series were in systemic CR when CNS-HL was discovered and 8 patients presented with CNS-HL, including 2 with isolated CNS disease.…”

“…The incidence has been reported as 0.2% to 0.5% of all HL cases, but a recent review of 14 868 patients identified only 2 cases of HL involving the CNS. [1][2][3] Most reports consist of single cases or small case series highlighting our limited knowledge about the clinical characteristics, prognosis, and management of this neurologic complication of HL. [4][5][6][7][8] Consequently, there is no consensus to guide therapeutic decision-making.…”

Intracerebral hogkin's lymphoma in a patient with human immunodeficiency virus

“…In the cases reported, as in ours, spinal fluid studies were not explanatory, and diagnosis required brain biopsies or was an autopsy finding. 5,6,7,8 Perhaps, the high risk of infection in these patients (including CNS), the use of empirical treatment, difficult diagnosis and poor outcome might have contributed to fewer cases being reported. A treatment strategy has not yet been defined in these patients.…”

Cerebrospinal Fluid Infiltration in Hodgkin Lymphoma

“…11 Treatment has varied extensively but includes whole brain radiation with or without combination chemotherapy. 1,12,13 The CSF cellular infiltrate clears following treatment with intrathecal methotrexate. 11 Nonetheless, most patients with direct invasion of brain, spinal cord or leptomeninges caused by Hodgkin lymphoma have a poor prognosis due to uncontrolled systemic tumor.…”

“…11 Nonetheless, most patients with direct invasion of brain, spinal cord or leptomeninges caused by Hodgkin lymphoma have a poor prognosis due to uncontrolled systemic tumor. 14 In a recent review by Hirmiz et al, 12 the prognosis is not dismal with appropriate treatment. However, in the index case reported, there was an initial good response clinically as well as cytologically, but, after 3 months, the patient died, consistent with the poor prognosis of CSF involvement by Hodgkin lymphoma.…”