Presentation of orbital solitary fibrous tumours

Abstract

Aims: To evaluate presenting features of patients with orbital solitary fibrous tumours (SFTs), based on histological phenotype.

Methods: A retrospective case-note review was performed for demographics and presenting features for patients with orbital SFTs. The tumours were classified as "Group IA" hypocellular SFT phenotype, "Group IB" haemangiopericytoma phenotype and low mitotic activity, and high-grade "Group II" haemangiopericytoma phenotype with high mitotic activity.

Results: Sixty-four patients (34 female; 53%) presented at a mean age of 42.2 years (median 38; range 19-82), with Group II patients presenting at an older age (mean 53 years). Median symptom duration was 12 months for Groups IA and IB, compared to 4 months for Group II, the commonest symptoms being proptosis (53%), diplopia (41%), periorbital swelling (31%), and altered vision (19%). Mean LogMAR was 0.17 (median 0.0; range -0.2-4), and 14% had ipsilateral optic neuropathy, with no significant difference between the three groups. Non-axial displacement was noted in 69%, a palpable mass in 45%, and reduced eye movements in 59%; choroidal folds and optic disc swelling were recorded in 12% and 9%. SFTs were mostly extraconal (59%), within the superior and superonasal quadrants (44%), with an average estimated tumour volume of 4.9 ml (median 3.6; range 0.31-14.5 ml).

Conclusion: SFTs may present with impaired visual function (∼15%), fundal abnormalities (a fifth), globe displacement (two-thirds), and reduced ocular motility (over a half). High-grade tumours tend to present more than a decade later, with a shorter duration of symptoms.

Fig. 1. Typical imaging for solitary fibrous tumours (SFTs).

A Non-contrast axial T1-weighted magnetic resonance image (MRI) showing a large right-sided isointense lateral intraconal mass, extending into an expanded superior orbital fissure, with (B) avid post-gadolinium contrast enhancement. C T2-weighted coronal MRI showing flow-void (arrow) within a superior extraconal SFT, and D post-contrast T1-weighted axial image of the same mass, shows enhancement of the lesion that contains a tortuous hypointense flow-void (arrow). E Coronal CT shows a superonasal quadrant SFT with remodelling of the orbital roof. F Axial MRI for a large, avidly enhancing superomedial SFT, with marked smooth deformation of the lamina papyracea.

Fig. 2

Estimated volume of tumour, related to the age at presentation for 56 patients with orbital solitary fibrous tumours (SFTs).