Solitary Fibrous Tumors of the Orbit and Central Nervous System: A Case Series Analysis.

Abstract

Introduction:Solitary fibrous tumor (SFT) is rarely diagnosed in clinical practice. Since its initial descriptions in the central nervous system (CNS) and the orbits, very few case reports and small case series have expanded their clinical and pathological characterization. We sought to describe a cases series of SFT from a single laboratory of neuropathology belonging to a tertiary university hospital.Methods:Retrospective clinical and histopathological description of eight cases of CNS and orbital SFT diagnosed over a 21-year period of time.Results:Median age was 47.3 years and four were males. Clinical presentation was related to local mass effect in all. Tumors occurred in the orbits (5/62.5%), intracranial dura attached (2), and the spinal medulla (1). The neuropathology showed the presence of hemangiopericytoma type (2), classic type (3), and mixed type (3). Histological anaplasia was present in two cases. Widespread/total immunoreactivity for vimentin, CD34, and Bcl-2 was present in all. Gross total removal was conducted in the majority (6/75%) and subtotal removal in 2 (25%). Three patients were submitted to adjuvant treatment (radiosurgery and radiotherapy). Recurrence occurred in four patients, 13–120 months after surgical intervention. Anaplasia was present in one case of recurrence.Conclusion:Our case series confirms the clinical and neuropathological diversity of CNS and orbital SFTs. Studies with longer follow-up periods are necessary to better understand the clinical behavior and prognosis of the SFT in the CNS and orbits.