Orbital solitary fibrous tumor: a clinicopathologic study from a Chinese tertiary hospital with a literature review

PURPOSE: To report the clinical features, imaging manifestations, histopathology, and immunohistochemical features of several cases of orbital solitary fibrous tumors (SFTs) in a Chinese tertiary hospital, and to undertake a literature review of this rare disease. METHODS: A non-comparative retrospective review of clinical presentations, imaging manifestations, histopathology, and immunohistochemical features as well as the management and disease outcomes of patients with orbital SFT was conducted along with a review of orbital SFT cases in the literature. RESULTS: This study includes two male and two female patients, with an average age of 53 years. Common presentations among these patients included a palpable subcutaneous mass, swelling of the eyelid, proptosis, diplopia, and vision disturbance. Three patients (cases 2–4) underwent imaging scans. All patients had complete surgical excisions and the tissue was subjected to pathological analysis. One patient (Case 4) experienced a recurrence with malignant transformation and received a re-excision surgery and postoperative radiotherapy. All patients remain alive and well after a minimum follow-up of 12 months (range 12–34 months). CONCLUSION: Despite its rare occurrence, we suggest that the possibility of orbital SFTs needs to be considered when a painless, slowly growing orbital mass is identified. Typical characteristic magnetic resonance imaging features of orbital SFTs are iso- or hypointense signals on T1 and T2-weighted images, with marked enhancement. A positive cluster of differentiation 34 (CD34) staining is an important diagnostic clue favoring SFT. Some orbital SFTs are infiltrating, aggressive, or recur with malignant transformation. Therefore, regular long-term follow-up after complete excision is mandatory.