A 66-year-old woman presented with dizziness and anemia. Brain MRI revealed an intradural lesion with adjacent vasogenic edema in the right frontal and parietal lobes with a dural tail sign and an arc-shaped homogenously enhancing mass in the subcutaneous soft tissue of the frontal region (Figure 1). Differential diagnosis for this lesion included meningioma, glioma, CNS lymphoma, or autoimmune disorders. Total resection of both lesions revealed pathological morphology consistent with lymphoplasmacytic lymphoma (Figure 2). Detection of an L265P mutation in the MYD88 gene in both CSF and bone marrow biopsy confirmed Bing-Neel syndrome, a rare neurological manifestation of Waldenstrom macroglobulinemia (WM).¹ The MYD88 gene encodes a vital protein involved in immune system signaling, with 95% of patients with WM exhibiting an L265P variation. The most common imaging findings are leptomeningeal infiltration or parenchymal involvement of the brain.² Hematological malignancy should be considered when a meningeal lesion is accompanied by an extracranial tumor.

一名 66 岁女性因头晕和贫血就诊。脑 MRI 显示右额叶和顶叶有硬膜内病变，邻近血管源性水肿，有硬脑膜尾征，额区皮下软组织有弧形均匀增强肿块（图 1）。该病变的鉴别诊断包括脑膜瘤、神经胶质瘤、中枢神经系统淋巴瘤或自身免疫性疾病。两个病灶的全切除显示病理形态与淋巴浆细胞淋巴瘤一致（图2）。在脑脊液和骨髓活检中检测到MYD88基因中的 L265P 突变证实了 Bing-Neel 综合征，这是华氏巨球蛋白血症 (WM) 的一种罕见的神经系统表现。¹ MYD88基因编码一种参与免疫系统信号传导的重要蛋白质，95% 的 WM 患者表现出 L265P 变异。最常见的影像学表现是软脑膜浸润或脑实质受累。²脑膜病变合并颅外肿瘤时应考虑血液系统恶性肿瘤。