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 extrapleural sites. These tumors are categorized as tumors of 'intermediate malignancy' under the World Health Organization classification of soft tissue tumors. 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

A giant orbital solitary fibrous tumor treated by surgical excision: a case report and literature review

“…Any orbital space, including the intraconal and extraconal spaces of the orbit, can be affected by orbital SFTs. Lesions occurring in other tissues, such as the conjunctivae, lacrimal gland fossa, lacrimal sacs, eyelid, and pigmented outer layer of the pars plana of the ciliary body, have also been reported [ 6 – 10 ].…”

Recurrent Orbital Solitary Fibrous Tumor in a 12-Year-Old

“…A review of the literature in 2003 described a total of 48 patients with orbital SFTs, only 3 of which were pediatric cases [5]. The youngest reported case occurred in a 5-year-old and two other pediatric recurrences have been reported in a 14-year-old girl and 15-year-old girl, respectively [6,7,8]. Patients with these tumors typically present with unilateral painless proptosis, eyelid swelling, a palpable mass, tearing, and/or blepharoptosis [3].…”