Matmati Kelly, Matmati Nabil, Hannun Yusuf A, Rumboldt Zoran, Patel Sunil, Lazarchick John, Stuart Robert, Giglio Pierre
Medical University of South Carolina, Charleston, SC, USA.
Hematol Rep. 2010 Jan 26;2(1):e10. doi: 10.4081/hr.2010.e10. Epub 2010 Dec 3.
Central nervous system (CNS) lymphoma involving the dura mater is very rare and histologically is usually a subtype of non-Hodgkin's lymphoma (NHL) termed mucosa-associated lymphoid tissue (MALT) lymphoma. We present a case of a 46-year old woman with dural MALT lymphoma that was found to also involve a lacrimal gland, inguinal lymph nodes, and bone marrow. Magnetic resonance imaging of the brain showed an extra-axial enhancing mass approximately 6 cm in maximum diameter along the right frontotemporal convexity. Histopathology of the resected dural mass showed MALT lymphoma expressing CD20, CD52, CD19, and CD38. Molecular studies of the B-cell receptor heavy chain demonstrated monoclonality at the involved sites. The patient was treated with four cycles of fludarabine, mitoxantrone, and rituximab with complete remission. She had recurrence in the subcutaneous tissue of the back at 12 months but has remained free of intracranial disease for 31 months. A review of the literature reveals 57 cases of dural MALT lymphoma. Only 4 had extra-CNS involvement at presentation, and only 3 had local recurrence of the dural tumor. Because of the indolent behavior of this tumor, the intracranial portion can be treated conservatively after resection with or without chemotherapy. Deferral of brain radiation can be considered with close clinical and neuroimaging follow up.
累及硬脑膜的中枢神经系统（CNS）淋巴瘤非常罕见，组织学上通常是非霍奇金淋巴瘤（NHL）的一种亚型，称为黏膜相关淋巴组织（MALT）淋巴瘤。我们报告一例46岁女性硬脑膜MALT淋巴瘤病例，该病例还累及泪腺、腹股沟淋巴结和骨髓。脑部磁共振成像显示，沿右侧额颞叶凸面有一个最大直径约6 cm的轴外强化肿块。切除的硬脑膜肿块的组织病理学检查显示MALT淋巴瘤表达CD20、CD52、CD19和CD38。对B细胞受体重链的分子研究表明，受累部位存在单克隆性。该患者接受了四个周期的氟达拉滨、米托蒽醌和利妥昔单抗治疗，完全缓解。她在12个月时背部皮下组织复发，但颅内疾病已缓解31个月。文献回顾显示有57例硬脑膜MALT淋巴瘤病例。仅4例在初诊时有CNS外受累，仅3例有硬脑膜肿瘤局部复发。由于该肿瘤生长缓慢，颅内部分在切除后可根据情况进行保守治疗，可联合或不联合化疗。在密切的临床和神经影像学随访下，可考虑推迟脑部放疗。
