Clinical characteristics and prognosis of orbital solitary fibrous tumor in patients from a Chinese tertiary eye hospital

Abstract

BACKGROUND Solitary fibrous tumor (SFT) is predominant within the pleura but very rare in the orbit, which is why the diagnosis of orbital SFT poses challenges in clinical practice. Accordingly, an integrated approach that incorporates specific clinical features, histological, histopathological, and immunohistochemical (IHC) examinations, and molecular analyses is warranted. AIM To retrospectively explore the clinical and imaging characteristics, treatment, outcomes of a series of patients with orbital SFT. METHODS We conducted a retrospective review of a series of patients diagnosed with a histopathologic orbital SFT treated at a single institution. All data on demogra/phics, clinical characteristics, imaging, treatment, postoperative histopathological and IHC examinations, and prognosis were collected. RESULTS In total, 13 patients were enrolled, 7 (53.8%) of whom had the tumor located in the superomedial quadrant of the orbit. Computed tomography revealed a solitary ovoid lesion in 10 (76.9%) patients and irregular lesion in 3 (23.1%) patients. Magnetic resonance imaging results were as follows: On T1 weighted images, 3 (23.1%) patients had hypointense mixed signals, whereas 10 (76.9%) patients showed isointense mixed signals; on T2 weighted images (T2WI), 3 (23.1%), 4 (30.8%), and 6 (46.2%) patients exhibited hypointense mixed, isointense mixed, and hyperintense signals, respectively. Notably, 12 (92.3%) patients showed significant enhancement, whereas there were patchy slightly enhanced areas in the tumor. All patients were treated by surgery. IHC analysis demonstrated that the tumor cells were immunoreactive for CD34, CD99, STAT-6, and vimentin in all patients. The lesions showed Ki-67 positivity < 5% in 1 (7.7) patient, 5%-10% in 10 (76.9%), and > 10% in 2 (15.4%). Two (15.4%) patients exhibited tumor recurrence. CONCLUSION The clinical manifestations and radiologic characteristics of orbital SFT are diverse and not specific. Accurate diagnosis and treatment require detailed radiological and histopathological/IHC evaluation.