Retrospective Study
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Sep 26, 2022; 10(27): 9670-9679
Published online Sep 26, 2022. doi: 10.12998/wjcc.v10.i27.9670
Clinical characteristics and prognosis of orbital solitary fibrous tumor in patients from a Chinese tertiary eye hospital
Ming-Yu Ren, Jing Li, Yi-Xiang Wu, Rui-Miao Li, Chi Zhang, Li-Min Liu, Jing-Jing Wang, Yu Gao
Ming-Yu Ren, Yi-Xiang Wu, Rui-Miao Li, Li-Min Liu, Jing-Jing Wang, Yu Gao, Department of Orbital Disease and Ocular Tumor, Hebei Eye Hospital, Xingtai 054001, Hebei Province, China
Jing Li, Intensive Care Unit, Xingtai Third Hospital, Xingtai 054001, Hebei Province, China
Chi Zhang, Department of Medical Imaging, Hebei Eye Hospital, Xingtai 054001, Hebei Province, China
Author contributions: Ren MY and Li J performed the research and wrote the paper; Ren MY and Li RM designed the research and supervised the report; Ren MY and Liu LM designed the research and contributed to the analysis; Wang JJ, Zhang C, and Gao Y provided clinical advice; Ren MY and Li J supervised the report; all authors revised the paper and approved the final version for submission.
Institutional review board statement: This study was reviewed and approved by the Ethics Committee of the Hebei Eye Hospital.
Informed consent statement: Patients were not required to give informed consent to the study because the analysis used anonymous clinical data that were obtained after each patient agreed to treatment by written consent.
Conflict-of-interest statement: All authors have no financial relationships to disclose.
Data sharing statement: Technical appendix, statistical code, and dataset available from the corresponding author at 147237583@qq.com. Participants gave informed consent for data sharing.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Ming-Yu Ren, MMed, Associate Chief Physician, Department of Orbital Disease and Ocular Tumor, Hebei Eye Hospital, No. 399 Quanbeidong, Xingtai 054001, Hebei Province, China. 147237583@qq.com
Received: May 27, 2022
Peer-review started: May 27, 2022
First decision: June 27, 2022
Revised: July 5, 2022
Accepted: August 21, 2022
Article in press: August 21, 2022
Published online: September 26, 2022
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 demographics, 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.

Keywords: Orbit, Solitary fibrous tumor, Diagnostic imaging, Immunohistochemistry, Surgical therapy

Core Tip: The clinical manifestations of orbital solitary fibrous tumor (SFT) are diverse and not specific. In most cases, the lesions occur outside the muscular cone, are localized at the superomedial quadrant and inferomedial quadrant of the orbit. The mean computed tomography values of lesions are variable, and the signal of lesions on magnetic resonance imaging is uncertain. Contrast-enhanced imaging showed that most part of the lesions was significantly enhanced, whereas there were patchy slightly enhanced areas in them. Delineating SFT from histologic mimics requires nuclear staining for STAT6 as a diagnostic adjunct in conjunction with CD34 positivity. Ki-67 labelling index may be extremely low, and malignant forms with an enhanced propensity for local recurrence have been reported.