Department of Neurosurgery, Washington University School of Medicine, Washington, D.C., USA.
Department of Pathology and Immunology, Washington University School of Medicine, Washington, D.C., USA.
World Neurosurg. 2021 Jun;150:179-196.e1. doi: 10.1016/j.wneu.2021.03.054. Epub 2021 Mar 18.
IgG4-related disease (IgG4-RD) is an inflammatory process that uncommonly can present in the skull base and calvarium and mimic a tumor but the nature of this condition is not well summarized in the neurosurgical literature.
A review was performed of 2 cases of IgG4-RD in the skull base highlighting the diagnostic challenges with assessment of these skull base lesions, and a systematic review of relevant literature was carried out.
A systematic review of the literature conducted in accordance with PRISMA guidelines identified 113 articles, with 184 cases of IgG4-RD in the skull base or calvarium. The most commonly affected locations include the meninges, cavernous sinus, base of the posterior fossa, clivus, and mastoid bone. Headache, visual and auditory disturbances, cranial nerve dysfunction, and seizures were the most common presenting symptoms. Medical treatment was highly successful and most commonly consisted of corticosteroids coadministered with immunosuppressive agents such as rituximab. Prevalence seemed to be equal between sexes, and serum IgG4 levels were increased in 61% of patients. Delayed diagnosis and a need for multiple biopsies were reported in numerous cases. Two cases of skull base IgG4-RD from the authors' institution show the variable presentations of this disease. More invasive surgical biopsies were required in both cases, and corticosteroid treatment led to significant clinical improvement.
IgG4-RD is an uncommon condition with an increasing body of reported cases that can affect the skull base and calvarium and should be in the differential diagnosis, because delay in diagnosis and treatment may be common.
IgG4 相关疾病（IgG4-RD）是一种炎症过程，罕见于颅底和颅骨，并可模拟肿瘤，但这种疾病的性质在神经外科学文献中尚未得到很好的总结。
对 2 例发生于颅底的 IgG4-RD 病例进行了回顾，强调了评估这些颅底病变时的诊断挑战，并对相关文献进行了系统回顾。
根据 PRISMA 指南进行的系统文献回顾确定了 113 篇文章，其中有 184 例 IgG4-RD 发生于颅底或颅骨。最常受累的部位包括脑膜、海绵窦、后颅窝底部、斜坡和乳突骨。头痛、视觉和听觉障碍、颅神经功能障碍和癫痫发作是最常见的表现症状。药物治疗非常成功，最常用的方法是联合使用皮质类固醇和免疫抑制剂，如利妥昔单抗。报道称，该病在性别之间的患病率相等，且 61%的患者血清 IgG4 水平升高。在许多病例中，报道了延迟诊断和需要多次活检的情况。作者机构的 2 例颅底 IgG4-RD 病例显示了这种疾病的多种表现。在这两种情况下，都需要进行更具侵袭性的外科活检，皮质类固醇治疗可显著改善临床症状。
IgG4-RD 是一种罕见疾病，其报道病例不断增加，可影响颅底和颅骨，应纳入鉴别诊断，因为延迟诊断和治疗可能很常见。
