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 con ned to the CNS, with cerebrospinal uid negative for cytology, is described in an immunocompetent patient previously treated for hyperthyroidism with 131 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 identi ed 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 unde ned. Although followup 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. Neuro-Oncology 2, 239-243, 2000 (Posted to Neuro-Oncology [serial online], Doc. 00-028, September 6, 2000 H odgkin's disease with CNS involvement is uncommon, even in the HIV-positive population, and usually occurs in the setting of advanced or recurrent disease. Reports of primary Hodgkin's disease conned to the CNS are exceedingly rare. We describe a case of primary CNS Hodgkin's disease in a patient previously treated with 131 I for Grave's hyperthyroidism and review the literature with respect to potential risk factors, management, and treatment outcomes.
Case StudyA 52-year-old right-handed male truck driver developed sudden onset of headache and confusion. He presented to the emergency department 2 days later with mild disorientation and receptive aphasia. Functional inquiry was negative for fatigue, weight loss, pruritis, fever, and night sweats. Physical examination revealed proptosis, but no evidence of peripheral adenopathy or hepatosplenomegaly. Assessment of cranial nerves, power, sensation, and coordination revealed no abnormalities.Past health included a history of Grave's hyperthyroidism with ophthalmopathy 4 years earlier. The patient was treated with radioactive iodine at a total dose of 229 mbq (6.2 mCi) administered orally. There was a 40-packyear smoking history . Medication on admission was limited to L-thyroxine, 0.125 mg per day. Family history was negative for cancer.Initial investigations revealed a normal complete blood cell count, peripheral blood smear, electrolytes, blood urea nitrogen, creatinine, random glucose, and international normalized ratio. Serum lactate dehydrogenase and erythrocyte sedimentation rate were not performed. A CT scan with contrast of the head revealed a contrast-enhancing 1.0 cm left temporoparietal lesion, with signi cant edema and mass effect (Fig. 1).