A review of solitary fibrous tumours of the orbit and ocular adnexa.

Abstract

Solitary fibrous tumour (SFT) is an uncommon spindle cell tumour of mesenchymal origin characterised by NAB2-STAT6 gene fusion. Although it was first described in the pleura, it can occur in connective tissue in any part of the body, but rarely presents in the orbit and ocular adnexa. SFT, which is part of the same disease spectrum as other fibroblastic tumours such as giant cell angiofibroma, haemangiopericytoma and fibrous histiocytoma, usually presents as a painless, slow-growing mass in any age group and generally follows a benign course, with a good prognosis after complete excision. However, malignant forms rarely occur. Even for benign tumours a more aggressive clinical behaviour is possible, with relentless infiltrative local growth, frequent recurrence following surgery, and malignant transformation with the potential for metastatic spread. Careful long-term follow-up is essential. The published literature on SFTs of the orbit and ocular adnexa is reviewed, and the aetiology, clinical presentation, epidemiology, radiological features, histopathology, immunohistochemistry, risk stratification, clinical management, and prognosis are discussed, reflecting on our own experience.