Meningeal solitary fibrous tumor as an unusual cause of expohthalmos: case report and review of the literature

Abstract

Objective and importance: We report an extremely rare case of a patient with meningeal solitary fibrous tumor with orbital involvement presenting as unilateral exophthalmos. This rare tumor should be considered in the differential diagnosis for aggressive dural-based lesions.

Clinical presentation: A 54-year-old man presented with a protruded eyeball on the right side and left hemiparesis. Computed tomography and magnetic resonance imaging demonstrated a huge mass, markedly enhanced in and around the right anterior clinoid process, which extended to the orbit and middle cranial fossa. Cerebral angiography revealed a richly vascular tumor fed by branches of both the right external and internal carotid arteries. A presumptive diagnosis of meningioma or hemangiopericytoma was considered.

Intervention: The tumor was exposed through an orbitozygomatic approach. At surgery, the tumor was grossly firm to hard and had destroyed the dura, orbital roof, anterior clinoid process, temporal bone, and muscle. Histological analysis revealed that the tumor was composed of spindle-cell proliferation in a collagen-rich background, but it exhibited regional variations. The vascular network demonstrated irregular vascular lumina with a "hemangiopericytoma-like" pattern. Histological features included high cellularity and a high degree of pleomorphism. Immunohistochemical analysis of the tumor demonstrated diffuse positive staining for CD34 and vimentin. The tumor displayed no positive staining for cytokeratin, epithelial membrane antigen, glial fibrillary antigenic protein, S-100 protein, and factor XIII.

Conclusion: Meningeal solitary fibrous tumor is considered a unique pathological entity. Wider use of immunohistochemical screening should enable analysis of the real incidence of these tumors; larger series and longer follow-up duration will allow conclusions to be drawn regarding treatment and prognosis. Differential diagnosis is discussed and the literature is reviewed.