Department of Neurosurgery, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan.
Department of Neurosurgery, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan.
World Neurosurg. 2019 Dec;132:69-74. doi: 10.1016/j.wneu.2019.08.127. Epub 2019 Aug 27.
Immunoglobulin (Ig)G4-related disease (IgG4-RD) was defined only recently and can be found in many organs. As intracranial lesions, hypophysitis and pachymeningitis are well known, whereas intracranial pseudotumor is unusual. This case involved multiple intracranial pseudotumors without extracranial lesions, mimicking multiple meningioma.
A 72-year-old woman was referred to our hospital with an incidental mass lesion at the craniocervical junction on magnetic resonance imaging (MRI). MRI showed diffuse enhanced extra-axial nodules around the medulla and middle cranial fossa. Surgery was performed for the gradually enlarging tumor. Intraoperative findings showed hard nodules around the vertebral artery. We performed subtotal resection. Neuropathological findings showed diffuse lymphoplasmacytic infiltration with lymphoid follicles. Immunohistochemical studies for IgG4 and IgG showed the histological criteria for IgG4-RD were met. Given the high IgG4 serum level, we diagnosed IgG4-RD. Steroid was administered initially but was then tapered, and nodules have since remained small.
This case demonstrates IgG4-RD mimicking meningioma with multiple masses but without extracranial lesions. This case and previous descriptions suggest the possibility of pseudotumor due to IgG4-RD in the presence of multiple extra-axial lesions at the dura and supplying artery, hard nodules with thickening of the artery, and frozen section findings of lymphoplasmacytes surrounded by rich collagen fibers. In such cases, the pseudotumor due to IgG4-RD should be resected, and total resection might not be warranted.
免疫球蛋白（Ig）G4 相关疾病（IgG4-RD）是最近才定义的，可发生于许多器官。作为颅内病变，垂体炎和硬脑膜炎较为常见，而颅内假瘤则不常见。本例涉及多个无颅外病变的颅内假瘤，类似于多发性脑膜瘤。
一名 72 岁女性因磁共振成像（MRI）显示颅颈交界处偶然出现肿块而被转至我院。MRI 显示延髓和中颅窝周围弥漫性强化的外轴结节。由于肿瘤逐渐增大，我们进行了手术。术中发现椎动脉周围有硬结节。我们进行了次全切除术。神经病理学检查显示弥漫性淋巴浆细胞浸润伴淋巴滤泡。免疫组化 IgG4 和 IgG 研究显示符合 IgG4-RD 的组织学标准。鉴于 IgG4 血清水平升高，我们诊断为 IgG4-RD。最初给予类固醇治疗，但随后逐渐减量，此后结节一直较小。
本例 IgG4-RD 表现为脑膜瘤样多发性肿块，但无颅外病变。本例及以往描述提示存在以下可能：硬脑膜和供血动脉的多个颅外病变、动脉增厚伴硬结节、富含胶原纤维的淋巴浆细胞围绕的冷冻切片发现，可能存在 IgG4-RD 引起的假性肿瘤。在这种情况下，应切除 IgG4-RD 引起的假性肿瘤，不一定需要完全切除。
