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

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 fluid negative for cytology, is described in an immunocompetent patient previousl y treated for hyperthyroidism with I-131, The patient underwent craniotomy, with resection of two lesions in dose proximity within the parenchyma of t he temporoparietal lobe. Histopathology revealed classic nodular sclerosing Hodgkin's disease, without evidence of Epstein-Barr viral infection, Treat ment included radiation to the whole brain with a boost to the tumor bed. T he patient made a full neurologic recovery and remains free of disease recu rrence 21 months after treatment. A literature review has identified only 9 additional cases. Seven of 8 evaluable patients remain alive and free of r ecurrence with a median follow-up of 13 months, The risk factors for this p resentation remain undefined. Although followup is short, radiotherapy alon e appears to provide excellent disease-free survival. Chemotherapy may be r eserved for patients with positive cerebrospinal fluid, extracranial diseas e, or subsequent relapse.