The Primary Brain Eosinophilic Angiocentric Fibrosis, A Rare Case Report

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.

A rare lumpy inflammatory lesion of the orbit: a unique case report

“…Histopathological examination is the main standard for diagnosing EAF. The typical pathological manifestations are massive infiltration of eosinophils, plasma cells, lymphocytes, and fibroblasts around small blood vessels; thickening of matrix; and characteristic rotation of onionskin-like collagen fibers and mesh proteins around occlusive blood vessels, accompanied by positive expression of IgG4 and IgG [ 1 , 2 ]. EAF should be distinguished from orbital inflammatory pseudotumor, hemangioma, and granulomatous disease.…”