Department of Neurosurgery, Xuanwu Hospital Capital Medical University, Beijing, China.
Chinese Pituitary Specialists Congress, Beijing, China.
Front Endocrinol (Lausanne). 2021 Apr 30;12:666791. doi: 10.3389/fendo.2021.666791. eCollection 2021.
IgG4-related inflammatory pseudotumors are very uncommon and are characterized histologically by the presence of inflammatory swellings with increasing IgG4-positive plasma cells and lymphocytes infiltrating the tissues. As reports of intracranial IgG4-related pseudotumors are very rare, we report a case of an IgG4-related inflammatory pseudotumor involving the clivus mimicking meningioma. A 46-year-old male presented with intermittent headache for 2 years and a sudden onset of dysphagia and dysphonia of 7 days' duration along with lower limb weakness. Enhanced magnetic resonance imaging (MRI) of the skull base revealed an isointense signal on T1- and T2-weighted images from an enhanced mass located at the middle of the upper clivus region, for which a meningioma was highly suspected. Then, an endoscopic transsphenoidal approach was adopted and the lesion was partially resected, as the subdural extra-axial lesion was found to be very tough and firm, exhibiting fibrous scarring attaching to the brain stem and basal artery. After the surgery, brain stem and posterior cranial nerve decompression was achieved, and the patient's symptoms, such as dysphagia, dysphonia and lower limb weakness, improved. Pathological findings showed many IgG4-positive plasma cells and lymphocytes surrounded by collagen-rich fibers. The patient was sent to the rheumatology department for further glucocorticoids after the diagnosis of an IgG4-related inflammatory pseudotumor was made. This case highlights the importance of considering IgG4-related inflammatory pseudotumors as a differential diagnosis in patients with lesions involving the clivus presenting with a sudden onset of symptoms of dysphagia and dysphonia along with lower limb weakness when other more threatening causes have been excluded. IgG4-related inflammatory pseudotumors are etiologically enigmatic and unpredictable, and total resection might not be warranted. Glucocorticoids are usually the first line of treatment after diagnosis.
IgG4 相关炎性假瘤非常罕见，其组织学特征为存在炎症性肿胀，伴有越来越多的 IgG4 阳性浆细胞和淋巴细胞浸润组织。由于颅内 IgG4 相关假性肿瘤的报道非常罕见，我们报告了一例累及斜坡的 IgG4 相关炎性假瘤，其表现类似于脑膜瘤。一名 46 岁男性因间歇性头痛 2 年，突发吞咽困难和声音嘶哑 7 天，伴下肢无力就诊。颅底增强磁共振成像（MRI）显示增强肿块位于中上斜坡区域，T1 和 T2 加权图像呈等信号，高度怀疑为脑膜瘤。然后，采用经蝶窦内镜入路部分切除病变，因为发现硬脑膜外轴外病变非常坚韧和牢固，纤维性瘢痕附着于脑干和基底动脉。手术后，实现了脑干和后颅神经减压，患者吞咽困难、声音嘶哑和下肢无力等症状得到改善。病理结果显示大量 IgG4 阳性浆细胞和淋巴细胞被富含胶原的纤维包围。诊断为 IgG4 相关炎性假瘤后，患者被转至风湿科接受进一步的糖皮质激素治疗。本例强调了在排除其他更具威胁性的原因后，对于累及斜坡、突然出现吞咽困难和声音嘶哑以及下肢无力等症状的患者，将 IgG4 相关炎性假瘤视为鉴别诊断的重要性。IgG4 相关炎性假瘤病因不明且不可预测，不一定需要完全切除。诊断后，糖皮质激素通常是一线治疗方法。
