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Journal of Clinical Oncology, Vol 25, No 11 (April 10), 2007: pp. 1437-1438 © 2007 American Society of Clinical Oncology. DOI: 10.1200/JCO.2006.10.1691
Hodgkin's Lymphoma Involving the CNSDepartment of Medicine, Lutheran General Hospital, Park Ridge, IL
Department of Pathology, Lutheran General Hospital, Park Ridge, IL
Oncology Specialists SC, Lutheran General Hospital Cancer Center, Park Ridge, IL 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 underwent right frontal craniotomy and resection of that lesion. The biopsy showed a dense mixed inflammatory infiltrate containing scattered 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. Additional 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, motor and/or sensory deficits, headaches, papilledema, coma, and seizures.2 Intracranial involvement is rare and presents as a late manifestation, usually with supratentorial predilection.4-6 The association 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, although some reports have suggested an increased involvement of CNS with mixed cellularity histology in up to 44% of cases reported.2,8 Investigators agree that patients who present with systemic disease are at higher risk for CNS involvement; however, most reported cases were found at the time of initial presentation as opposed to our patient who was in CR at the time of his CNS relapse. Mechanisms that could explain intracerebral metastases in this setting include direct spread from dural and/or osseous deposits, from leptomeningeal metastases, or by direct extension from paracranial or paraspinal lymph node involvement. Hematogenous path originating in lymph nodes is another plausible explanation.9,10 In an attempt to explain the rarity of intracranial involvement of HL, Ogata et al suggested that the lack of extravasation of Reed-Sternberg cells into the perivascular space, commonly present in non-Hodgkin's lymphoma, could be due to a filtering effect of the lungs or to the intrinsic characteristics of the cells themselves. Their large size could preclude them from permeating the CNS.11,12 The rarity of intracerebral metastases argues for tissue diagnosis in all conditions to exclude other possibilities, such as primary brain tumors or non-Hodgkin's lymphoma. Primary CNS HL has been reported in few cases, one of which is associated with Epstein-Barr virus.13 There is no clear consensus as to the best treatment of patients with CNS involvement, but radiotherapy has been the standard approach. Despite aggressive treatment, the prognosis remains poor, with a median time from diagnosis to death of 46 months.3 It remains plausible, although unlikely, that our patient had occult asymptomatic CNS involvement at the time of his initial diagnosis. The fact that his CNS disease was discovered at the time where he was in CR by all imaging modalities, makes this case a unique and a rare event. AUTHORS' DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The authors indicated no potential conflicts of interest.
REFERENCES
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Copyright © 2007 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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