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Primary Hodgkin's disease of the CNS in an immunocompetent patient: A case study and review of the literature

Divisions of Medical Oncology and Hematology (J.B., N.B.), Radiation Oncology (R.G.M.), and Pathology (M.S.L., M.S.), Toronto - Sunnybrook Regional Cancer Centre, Sunnybrook and Women's College Health Science Centre, University of Toronto, Toronto, Ontario, Canada M4N 3M5

¹ Address correspondence and reprint requests to Robert G. MacKenzie, Division of Radiation Oncology, Toronto-Sunnybrook Regional Cancer Centre, 2075 Bayview Ave., Toronto, Ontario, Canada, M4N 3M5.

Abstract

Primary Hodgkin's disease limited to the CNS is exceedingly rare. Little is known regarding etiologic risk factors, optimal management, and prognosis. A case of Hodgkin's disease confined to the CNS, with cerebrospinal `uid negative for cytology, is described in an immunocompetent patient previously treated for hyperthyroidism with <sup>131</sup>I. The patient underwent craniotomy, with resection of two lesions in close proximity within the parenchyma of the temporoparietal lobe. Histopathology revealed classic nodular sclerosing Hodgkin's disease, without evidence of Epstein-Barr viral infection. Treatment included radiation to the whole brain with a boost to the tumor bed. The patient made a full neurologic recovery and remains free of disease recurrence 21 months after treatment. A literature review has identified only 9 additional cases. Seven of 8 evaluable patients remain alive and free of recurrence with a median follow-up of 13 months. The risk factors for this presentation remain undefined. Although follow-up is short, radiotherapy alone appears to provide excellent disease-free survival. Chemotherapy may be reserved for patients with positive cerebrospinal `uid, extracranial disease, or subsequent relapse.

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				B. E. Figueroa, J. R. Brown, A. Nascimento, D. C. Fisher, and S. Tuli Unusual Sites of Hodgkin's Lymphoma: CASE 2. Hodgkin's Lymphoma of the CNS Masquerading As Meningioma J. Clin. Oncol., October 15, 2004; 22(20): 4228 - 4230. [Full Text] [PDF]