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© 2003 American Society for Clinical Oncology
Patients Presenting With CNS LesionsCASE 1. PRIMARY LOW-GRADE MUCOSA-ASSOCIATED B-CELL LYMPHOMA OF THE DURAHospital Felício Rocho, Belo Horizonte, Minas Gerais, Brazil
A 36-year-old black woman presented with a history of generalized headache during the previous 3 years, and episodes of generalized tonic-clonic seizures occurring in the past month. Magnetic resonance imaging revealed a neoplastic lesion involving the meninges bilaterally on the parietal convexity, the posterior region of the falx cerebri, and the cerebellar tentorium to the right (Fig 1
CNS primary lymphomas (CNSPLs) are rare diseases totaling between 0.5% to 1.2% of intracranial neoplasms. There is a usual association with immunodefficiency states, though a considerably increased incidence in immunocompetent individuals has been observed. Leptomeningeal primary lymphomas with no associated cerebral parenchymal lesions are uncommon, compounding approximately 7% of CNSPLs.1 There are few cases of leptomeningeal involvement by low-grade B-cell lymphoma reported in literature. The histologic aspects and clinical behavior of such cases suggest that these tumors may belong to the mucosa-associated lymphoma (MALT) subgroup.2,3 MALT lymphomas are considered to originate from marginal zone B-lymphocytes, and are characterized by the presence of "small centrocyte-like cells" or monocytoid B-cells. They are associated with reactive germinal center formation and may contain numerous large, transformed lymphocytes; reactive or neoplastic plasma cells; and other inflammatory cells. Immunophenotypically, they express B-cell associated antigens such as CD20 and CD79a, with no expression of CD5, CD10, or CD 23.3,4 CNS MALT lymphomas occur more frequently in females. Patients present with headache, visual disturbances, and seizures. Differential diagnoses are made mostly with meningiomas, based on radiological findings.2,3 MALT lymphomas of the dura have been treated with a variety of therapeutic options, including radiotherapy, systemic chemotherapy, and partial surgical excision.5–8 The recognition and diagnosis of this condition has important therapeutic implications, as MALT lymphomas respond well to local treatment and have a better prognosis than other CNSPLs. AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The authors indicated no potential conflicts of interest.
REFERENCES 1. DeAngelis LM, Yahalom J: Primary central nervous system lymphoma, in DeVita VT Jr (ed): Cancer Principles and Practice of Oncology. Philadelphia, PA, Lippincott Williams and Wilkins, 2001, pp 2330–2338 2. Kumar S, Kumar D, Kaldjian EP, et al: Primary low-grade B-cell lymphoma of the dura: A mucosa associated lymphoid tissue-type lymphoma. Am J Surg Pathol 21:81–87, 1997 (suppl 1)[CrossRef][Medline] 3. Kambham N, Chang Y, Matsushima AY: Primary low-grade B-cell lymphoma of mucosa associated lymphoid tissue (MALT) arising in the dura. Clin Neuropathol 17:311–317, 1998[Medline] 4. Armitage JO, Weisenburger DD: New approach to classifying non-Hodgkin’s lymphoms: Clinical features of the major histologic subtypes. J Clin Oncol 16:2780–2795, 1998[Abstract] 5. Connors JM: Problems in lymphoma management: Special sites of presentation. Oncology 12:185–195, 1998[Medline] 6. Abrey LE, DeAngelis LM, Yahalom J: Long term survival in primary CNS lymphoma. J Clin Oncol 16:859–863, 1998[Abstract]
7. DeAngelis LM, Yahalom J, Thaler HT, et al: Combined modality therapy for primary CNS lymphoma. J Clin Oncol 10:635–643, 1992
8. Abrey EB, Yahalom J, DeAngelis LM: Treatment for primary CNS lymphoma: The next step. J Clin Oncol 18:3144–3150, 2000
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Copyright © 2003 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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