Hodgkin’s Lymphoma Involving the CNS

A 51-year-old man with Down syndrome was diagnosed with stage IVB mixed-cellularity Hodgkin’s lymphoma (HL). He was treated with adriamycin, bleomycin, vinblastine, and dacarbazine, and attained complete remission (CR) by virtue of negative bone marrow biopsies, complete resolution of his adenopathy, and a negative positron emission tomography scan uptake after the fourth cycle. The patient was planned to undergo a total of six cycles, but several days after completion of his fifth cycle he experienced a witnessed tonic-clonic seizure. A T1 magnetic resonance imaging of the brain with gadolinium showed a 1.5 cm enhancing mass with surrounding edema in the right frontal lobe with a mass effect on the right lateral ventricle and slight bowing of the midline (Fig 1). That same mass appeared hypointense on the magnetic resonance imaging T2 weighted modality (Fig 2), suggestive of lymphoma. The patient un-derwent right frontal craniotomy and resection of that lesion. The biopsy showed a dense mixed inflammatory infiltrate containing scat-tered large binucleate CD30 and Fascin positive consistent with Reed-Sternberg cells, diagnostic of HL (Figs 3A, 3B, and 4). Radiation therapy started and the patient experienced no further seizures. Addi-tional staging studies failed to reveal a recurrence of the disease outside the CNS. He continues to be in remission. HL involving the CNS is a rare event occurring in 0.2 % to 0.5% of cases.1-3 Common presentations include cranial nerve palsies, mo-tor and/or sensory deficits, headaches, papilledema, coma, and sei-zures.2 Intracranial involvement is rare and presents as a late manifestation, usually with supratentorial predilection.4-6 The associ-ation between Down syndrome and HL is uncommon with only six cases reported to date, most occurring in young males.7 Risk factors for intracranial HL have not been determined, al-though some reports have suggested an increased involvement of CNS with mixed cellularity histology in up to 44 % of cases reported.2,