A Rare Case of a Recurrent Giant Solitary Fibrous Tumor of the Ciliary Body of the Orbit.

Abstract

Solitary fibrous tumors (SFTs) are uncommon spindle-cell tumors of mesenchymal origin initially described in the pleura and subsequently in other extra-pleural sites. These tumors are categorized as tumors of 'intermediate malignancy' under the World Health Organization classification of soft tissuetumors. SFT was virtually non-existent or misdiagnosed until its characteristic features, particularly the strong and diffuse immuno-reactivity to CD 34 were described. Extra-pleural manifestations of SFT, particularly in the head and neck region are rare. Although a number of isolated case reports of orbital SFTs have been described ever since its initial description in 1994, cases of recurrent SFTs have been very few. Recurrences of these tumors following surgery are considered unusual and metastasis exceptional. We describe clinical presentation and the management challenges of recurrent giant orbital SFT in a 15-year old girl along with a brief review of literature.