title: Primary central nervous system mucosa-associated lymphoid tissue lymphoma: case report and literature review.
abstract: Primary presentation of intradural non-Hodgkin lymphoma is rare. Recently, B-cell lymphomas of mucosa-associated lymphoid tissue (MALT) have been recognized as an important pathologic subtype. When MALT lymphomas present in the central nervous system (CNS), they are distinguishable from primary high-grade CNS lymphomas. We present the clinicopathologic features of 5 patients with primary CNS MALT lymphoma treated at our institution from 1999 to 2006. Four out of 5 patients were women, and all patients presented with headaches, focal motor deficits, or cranial nerve palsy. Radiologic studies demonstrated ill-defined dural masses in 3 and well-defined masses in 2 patients. Pathology revealed small to medium-sized cells with a moderate amount of cytoplasm and irregular nuclear borders, expressing pan B-cell markers (CD19, CD20, and CD79a) but lacking CD10, CD23, and cyclin D1, confirming low-grade MALT lymphoma. Plasma cells were encountered in all the biopsies with variable reactive T-cell infiltration. wedge chain restriction was seen in 3 patients. Therapy consisted of either surgical resection, whole-brain radiation, or systemic or intrathecal chemotherapy. There was no evidence of recurrence or systemic relapse in 4 patients at 4 years of follow-up. One patient died in 2 months, unrelated to CNS lymphoma. This case series illustrates the rare occurrence of low-grade dural B-cell lymphoma and the need to consider this entity in the differential diagnosis of CNS lesions.
authors.affiliation: Department of Medicine, Division of Hematology/Oncology, St Luke's Roosevelt Hospital Center, New York, NY Continuum Cancer Center of New York, NY 10019, USA.
authors.forename: Wajeeha
authors.initials: W
authors.lastname: Razaq
authors.forename: Anupama
authors.initials: A
authors.lastname: Goel
authors.forename: Ali
authors.initials: A
authors.lastname: Amin
authors.forename: Michael L
authors.initials: ML
authors.lastname: Grossbard
mesh_terms: D000368:Aged; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents / Q000627:therapeutic use; D016543:Central Nervous System Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000628:therapy*; D002893:Chromosomes, Human, Pair 3 / Q000648:ultrastructure; D005260:Female; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D016393:Lymphoma, B-Cell / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000628:therapy*; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000628:therapy*; D008297:Male; D008579:Meningioma / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D008875:Middle Aged; D012008:Recurrence; D000069283:Rituximab; D014314:Trisomy
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D000069283:Rituximab