• Hind Manaa Alkatan, Abrar K Alsalamah, Abdulrahman Almizel, Khalid M Alshomar, Azza My Maktabi, Sahar M ElKhamary, Charles G Eberhart, Adriana Iuliano, Vittoria Lanni, and Diego Strianese.
• From the Department of Ophthalmology, King Saud University, Riyadh, Saudi Arabia.
• Ann Saudi Med. 2020 May 1; 40 (3): 227-233.

BackgroundSolitary fibrous tumors (SFT), formerly called hemangiopericytoma, are rare tumors derived from mesenchymal cells originally described in the pleura, but these tumors may affect extraserosal tissues including the lacrimal gland and orbit.ObjectiveConduct a multi-centered clinical, radiological and histopathological analysis of 17 orbital SFT cases.DesignA retrospective case series.SettingThree eye centers in two countries.Patients And MethodsThe data collected from the charts of 17 adult patients presenting with tissue diagnosis of orbital hemangiopericytoma or SFT from January 2003 to December 2018 included demographics, clinical imaging and histopathological information including immunohistochemical (IHC) characteristics.Main Outcome MeasuresThe demographic characteristics, clinical presentation, and histopathological patterns or variants of SFT were analyzed.Sample Size17 adult patients.ResultsMean age was 45 years (range 23-80 years). Male to female ratio was 3:1. The right eye was affected in 12 (70.5%) patients. Commonest presentation was proptosis in 13/17 (76% of patients). Other symptoms were impaired motility (29%) and ptosis (11%). Lesions mostly affected the medial orbit (35%), then orbital apex in 11%. The histopathological classic pattern-less variant was the commonest. One case with aggressive behavior, multiple recurrences and atypical features was encountered. Immunohistochemical (IHC) markers used included CD34 expression in all cases, Bcl-2 expression in 10/11, CD99 in 9/9 and Vimentin in 4/4. STAT6 was used in 2 cases.ConclusionsSFTs are rare tumors affecting the orbit in both genders equally in their mid-forties, but showed male predominance in our analysis with a predominant classic histopathological pattern. Tissue diagnosis is essential and requires IHC studies for confirmation.LimitationsSample size is relatively small owing to the rarity of this tumor in the orbit.Conflict Of InterestNone.

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