Lymphomatoid granulomatosis (LYG) is a rare lymphoproliferative disease with angiocentric and angiodestructive infiltrates, and by definition, Epstein-Barr virus (EBV)-associated B-cell malignancy. It most frequently involves the lung, and in some cases, the lesions are confined to the central nervous system (isolated CNS-LYG). However, it remains a controversial disease in terms of pathophysiology, especially in those confined to the CNS. We report the case of a 37-year-old man with CNS lesion pathologically characterized by angiocentric, T-cell-rich lymphoid cell infiltrates that resembled CNS-LYG. The lesion was clinically aggressive with subacute onset and irregular ring-like enhancement on MRI. The resected specimen showed no cytological atypia, EBV-infected cells, or monoclonality for IgH and TCR gene rearrangements. Considering the possibility of latent malignancy, the patient was successfully treated with corticosteroid and chemoradiotherapy with high-dose methotrexate. The present case and the literature suggest that EBV-negative CNS lesions with angiocentric lymphoid infiltrates are probably heterogeneous in their pathogenesis, including those that could fit into the so-called CNS-LYG and those with T-cell predominance. The accumulation of similar cases is warranted for the classification and appropriate treatment of these lesions.

淋巴瘤样肉芽肿病 (LYG) 是一种罕见的淋巴组织增生性疾病，具有血管中心性和血管破坏性浸润，并且根据定义，与 EBV 相关的 B 细胞恶性肿瘤。它最常累及肺，在某些情况下，病变局限于中枢神经系统（孤立的 CNS-LYG）。然而，就病理生理学而言，它仍然是一种有争议的疾病，特别是在那些局限于中枢神经系统的疾病中。我们报告了一例 37 岁男子的 CNS 病变，其病理特征为血管中心性、富含 T 细胞的淋巴细胞浸润，类似于 CNS-LYG。病变在临床上具有侵袭性，在 MRI 上呈亚急性起病和不规则环状增强。切除的标本未显示细胞学异型性、EBV 感染细胞或 IgH 和 TCR 基因重排的单克隆性。考虑到潜在恶性肿瘤的可能性，患者接受了糖皮质激素和大剂量甲氨蝶呤放化疗的成功治疗。本病例和文献表明，伴有血管中心淋巴浸润的 EBV 阴性 CNS 病变的发病机制可能具有异质性，包括那些可能适合所谓的 CNS-LYG 的病变和具有 T 细胞优势的病变。类似病例的积累对于这些病变的分类和适当治疗是必要的。包括那些可以适应所谓的 CNS-LYG 的和那些以 T 细胞为主的。类似病例的积累对于这些病变的分类和适当治疗是必要的。包括那些可以适应所谓的 CNS-LYG 的和那些以 T 细胞为主的。类似病例的积累对于这些病变的分类和适当治疗是必要的。