Kim Joong-Yub, Jung Kyeong Cheon, Park Sung-Hye, Choe Ji-Young, Kim Ji Eun
Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.
Department of Pathology, Seoul National University, College of Medicine, Seoul, Korea.
Neuropathology. 2018 Apr 10. doi: 10.1111/neup.12467.
Lymphomatoid granulomatosis (LYG) is a rare lymphoproliferative disorder characterized by infiltration of Epstein-Barr virus (EBV)-positive large atypical B-cells in an angiocentric fashion in a mixed inflammatory background. The histologic spectrum of LYG ranges from reactive proliferation to diffuse large B-cell lymphoma according to the number of EBV+ B-cells. It is known that virtually all patients have pulmonary involvement, whereas primary LYG of the other organs has been rarely reported. Herein, we describe three cases of primary LYG of the central nervous system (CNS) without pulmonary lesions, and this is the first collection to be reported in Korea. All of the cases revealed multifocal enhancing necrotic brain lesions masking as metastatic tumors, infection or vasculitis. These patients were successfully managed by corticosteroids and immunomodulating agents without chemotherapy against malignant lymphoma even in grade 3 LYG. We assume that primary CNS LYG might be less aggressive and more controllable than pulmonary LYG. The clinicopathologic characteristics of the cases with a special regard to the differential diagnosis and clinical courses are discussed in combination with an overview of the literature.
淋巴瘤样肉芽肿病（LYG）是一种罕见的淋巴增殖性疾病，其特征是在混合性炎症背景下，爱泼斯坦-巴尔病毒（EBV）阳性的大异型B细胞以血管中心性方式浸润。根据EBV+B细胞的数量，LYG的组织学谱从反应性增生到弥漫性大B细胞淋巴瘤不等。已知几乎所有患者都有肺部受累，而其他器官的原发性LYG很少有报道。在此，我们描述了3例无肺部病变的中枢神经系统（CNS）原发性LYG病例，这是韩国首次报道的此类病例集。所有病例均显示多灶性强化坏死性脑病变，类似转移性肿瘤、感染或血管炎。即使是3级LYG患者，这些患者通过皮质类固醇和免疫调节剂成功治疗，无需针对恶性淋巴瘤进行化疗。我们推测原发性中枢神经系统LYG可能比肺部LYG侵袭性更小、更可控。结合文献综述，讨论了这些病例的临床病理特征，特别关注鉴别诊断和临床过程。
