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Neuro-Oncology 2000 2(4):239-243; doi:10.1093/neuonc/2.4.239
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© 2000 by the Society forNeuro-Oncology

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

Jim Biagi, Robert G. MacKenzie1, Megan S. Lim, Michelle Sapp and Neil Berinstein

Divisions of Medical Oncology and Hematology (J.B.,N.B.), Radiation Oncology (R.G.M.), and Pathology (M.S.L., M.S.), Toronto - Sunnybrook RegionalCancer 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, Divisionof Radiation Oncology, Toronto-Sunnybrook Regional Cancer Centre, 2075 BayviewAve., Toronto, Ontario, Canada, M4N 3M5.


  Abstract

Primary Hodgkin's disease limited to the CNS is exceedingly rare. Little isknown regarding etiologic risk factors, optimal management, and prognosis. Acase of Hodgkin's disease confined to the CNS, with cerebrospinal `uidnegative for cytology, is described in an immunocompetent patient previouslytreated for hyperthyroidism with 131I. The patient underwentcraniotomy, with resection of two lesions in close proximity within theparenchyma of the temporoparietal lobe. Histopathology revealed classicnodular sclerosing Hodgkin's disease, without evidence of Epstein-Barr viralinfection. Treatment included radiation to the whole brain with a boost to thetumor bed. The patient made a full neurologic recovery and remains free ofdisease recurrence 21 months after treatment. A literature review hasidentified only 9 additional cases. Seven of 8 evaluable patients remain aliveand free of recurrence with a median follow-up of 13 months. The risk factorsfor 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.

Received April 18, 2000; Accepted June 19, 2000


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