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Journal of Clinical Oncology, Vol 22, No 20 (October 15), 2004: pp. 4228-4230 © 2004 American Society of Clinical Oncology. DOI: 10.1200/JCO.2004.01.163
Unusual Sites of Hodgkin's LymphomaCASE 2. Hodgkin's Lymphoma of the CNS Masquerading As MeningiomaBrigham and Women's Hospital, Dana-Farber Cancer Institute, Harvard Medical School, Brookline,MA A 23-year-old healthy woman presented with a month-long history of persistent headache of increasing severity. She noted episodic nausea and vomiting in association with her headaches that were exacerbated by exertion. Family history was remarkable for a paternal aunt who died at age 23 years of Hodgkin's lymphoma. Physical examination revealed nystagmus with bidirectional lateral gaze and left upper-extremity dysmetria. Magnetic resonance imaging of the brain demonstrated a left-sided, extra-axial posterior fossa mass that was broadly based on the retrosigmoid dura, and enhanced homogeneously (Fig 1). Imaging characteristics were consistent with meningioma.
The patient underwent a posterior fossa craniectomy and tumor excision without complication. Pathologic evaluation demonstrated a fibrous tumor composed of small lymphocytes and scattered plasma cells consistent with nodular sclerosis type Hodgkin's lymphoma (Fig 2). Reed-Sternberg cells were present (Fig 2, arrow) and were positive for CD15 (Fig 3) and CD30 (Fig 4) by immunoperoxidase staining. In situ hybridization for Epstein-Barr virus–associated RNA was negative (Figure not shown). The patient underwent computed tomography–assisted staging, neuroaxis magnetic resonance imaging, lumbar puncture, and bone marrow biopsy. The studies demonstrated prevascular, pretracheal, and supraclavicular lymphadenopathy; a T10 vertebral body metastasis with adjacent epidural enhancement; normal CSF protein and negative CSF cytology; and negative bone marrow pathology. Serologic testing for HIV was negative. The patient's stage IV Hodgkin's lymphoma has been subsequently treated with six cycles of chemotherapy with doxorubicin, bleomycin, vinblastine, dacarbazine (ABVD), with four treatments of intrathecal thiotepa, followed by 36 Gy radiotherapy to the posterior fossa, and 30.6 Gy to the mediastinum, including T10. The patient entered complete remission after three cycles of ABVD and remains in ongoing complete remission.
Hodgkin's disease (now referred to as Hodgkin's lymphoma by the WHO) involves the CNS in 0.2% to 0.5% of cases either by contiguous or hematogenous spread.1 CNS involvement is more common in patients with widespread relapsed disease2 or at initial diagnosis in immunocompromised patients, but can also be seen at initial diagnosis in immunocompetent patients, as in this case. Parenchymal and leptomeningeal spread have both been reported.1,3-6 Although intraparenchymal lesions are more frequent, dural-based masses that resemble meningioma have been reported in primary and secondary Hodgkin's lymphoma of the CNS.3,5,6 Etiology, treatment, and prognosis have not been established.7 Median survival following local (10 to 20 Gy) and whole-brain (30 to 40 Gy) radiotherapy has been reported in the range of 8 months to 2 years.3-5 Systemic chemotherapy in combination with radiotherapy has been advocated in the presence of positive CSF cytology or extracranial involvement. Treatment protocols have included ABVD, COPP (cyclophosphamide, vincristine, procarbazine, prednisone), MOPP (nitrogen mustard, vincristine, procarbazine, prednisone), and/or intrathecal methotrexate.2,5,7Long-term disease-free survival has been reported following combined-modality therapy.2,5,7
Authors' Disclosures of Potential Conflicts of Interest
REFERENCES 1. Ashby MA, Barber PC, Holmes AE, et al: Primary intracranial Hodgkin's disease. Am J Surg Pathol 12:294-299, 1988[Medline]
2. Anselmo AP, Proia A, Cartoni C, et al: Meningeal localization in a patient with Hodgkin's disease: Description of a case and review of the literature. Ann Oncol 7:1071-1075, 1996 3. Biagi J, MacKenzie RG, Lim MS, et al: Primary Hodgkin's disease of the CNS in an immunocompetent patient: A case study and review of the literature. Neuro-oncol 2:239-243, 2000[Abstract] 4. Johnson MD, Kinney MC, Scheithauer BW, et al: Primary intracerebral Hodgkin's disease mimicking meningioma: Case report. Neurosurgery 47:454-456, 2000[CrossRef][Medline] 5. Klein R, Mullges W, Bendszus M, et al: Primary intracerebral Hodgkin's disease. Am J Surg Pathol 23:477-481, 1999[CrossRef][Medline] 6. Nakayama H, Tokuuye K, Kagami Y, et al: Brain involvement in Hodgkin's disease: Case reports and review of the literature. Radiat Med 18:205-208, 2000[Medline] 7. Sapozink MD, Kaplan HS: Intracranial Hodgkin's disease: A report of 12 cases and review of the literature. Cancer 52:1301-1307, 1983[CrossRef][Medline]
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Copyright © 2004 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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