Abstract：
Eosinophilic angiocentric fibrosis (EAF) is a rare progressive fibrosing lesion involving the nasal cavity, paranasal sinuses, and the upper respiratory tract. There are few reports that it rarely involves the orbit; however, there is no report of intracranial involvement. Here, we report and share our experience with a rare case of primary intracranial EAF. A 33-year-old woman with a history of a suprasellar mass and unsuccessful surgical and medical treatment referred to us. Physical examination demonstrated right-sided blindness and ptosis, left-sided decreased visual acuity, and visual field defect. The brain imaging revealed an extra-axial intradural well-defined large suprasellar mass with parasellar (more on the right side) and retrosellar extension. Via pterional craniotomy and subfrontal approach, a very firm creamy-brownish well-defined fibrotic mass was encountered. The tumour texture was too firm to be totally resected. The microscope exited the surgical field off, and the tumour was incompletely resected using a rongeur. The histopathology finding favoured EAF. Further histopathology evaluation failed to show histologic features of IgG4-related disease. Although the preoperative diagnosis of EAF is impossible, in the setting of an indolent slow-growing lesion demonstrating hypointensity on the T2 image sequence of MRI (magnetic resonance imaging), EAF should be considered a differential diagnosis. In the setting of this diagnosis, the systemic and other organ involvement for a diagnosis of IgG4-RD should be evaluated. However, more cases are needed to illustrate the relation between these two entities.
摘要：
嗜酸性粒细胞性血管中心纤维化（EAF）是一种罕见的累及鼻腔的进行性纤维化病变，鼻旁窦,和上呼吸道。很少有报道说它很少涉及轨道；然而，没有颅内受累的报告.这里,我们报告并分享了罕见的原发性颅内EAF病例的经验。一位33岁的女性，有鞍上肿块的病史，手术和药物治疗失败。体格检查显示右侧失明和上睑下垂，左侧视力下降，和视野缺陷。脑成像显示轴外硬膜内清晰的大的鞍上肿块，具有鞍旁（右侧更多）和鞍后延伸。通过翼点开颅术和额叶下入路，一个非常坚定的奶油褐色明确定义的纤维化肿块。肿瘤质地太硬，无法完全切除。显微镜离开了手术区，而肿瘤是用骨钳切除的.组织病理学发现有利于EAF。进一步的组织病理学评估未能显示IgG4相关疾病的组织学特征。虽然EAF的术前诊断是不可能的,在MRI（磁共振成像）的T2图像序列上显示低张力的惰性缓慢生长病变的背景下，EAF应被视为鉴别诊断。在这种诊断的设置中，应评估用于诊断IgG4-RD的全身和其他器官受累情况.然而,需要更多的案例来说明这两个实体之间的关系。
