Aims Adenomas of the ciliary body epithelium, including adenoma of the pigmented ciliary body epithelium (APCE) and adenoma of the non-pigmented ciliary body epithelium (ANPCE), are extremely rare, and most knowledge about them comes from sporadic case reports. The purpose of this study was to provide a comprehensive understanding of adenomas of the ciliary body epithelium and to identify the similarities and differences between APCE and ANPCE.
Methods This study was a retrospective case series comprising data from 41 patients obtained from retrieved publications and five cases diagnosed at the Shanghai Ninth People’s Hospital. The clinicopathological features, treatment and prognosis of APCE and ANPCE were compared using the non-parametric rank sum test, t-test and the χ2 test.
Results The clinical and histopathological features and treatment were analogous between APCE (n=23) and ANPCE (n=23). The overall visual prognosis associated with the two tumours was good, with 63% of the patients having stable or improved vision after treatment. Enucleation was the primary cause of eventual vision loss (three in APCE vs two in ANPCE, p=0.001). Notably, iris invasion was commonly observed in patients with APCE (six in APCE vs zero in ANPCE, p=0.014), and iris invasion was associated with decreased vision eventually (p=0.003). Tumour size was irrelevant to the vision outcome (p=0.65). Metastasis or recurrence did not occur in any of the patients.
Conclusion In most cases, the clinicopathological features of ANPCE and APCE were similar. Iris invasion was commonly observed in patients with APCE, which was associated with poor visual prognosis.
- ciliary body
- treatment other
Data availability statement
Data are available upon reasonable request. Data are available on reasonable request. The datasets that support the findings of this study are available from the corresponding author upon reasonable request.
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YZ, XG and YY are joint first authors.
YZ, XG and YY contributed equally.
Contributors AZ designed the study. XX and RJ provided the data of patients. YZ, XG and YY collected, analysed and interpreted the data. HP and YY performed the literature search. YZ wrote the initial draft. AZ and XG revised the manuscript. AZ, XX, and RJ supervised the study. AZ was the guarantor. All authors provided a final review and approved the manuscript before submission.
Funding The work was supported by the Cross-disciplinary Research Fund of Shanghai Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine (JYJC202210 and YG2023QNB15), Shanghai Science and Technology Commission Research Grant (20DZ2270800, 23ZR1438400 and 23YF1422400), Shanghai Key Clinical Specialty, Shanghai Health Commission (2022ZZ01003), the Project of Biobank of Shanghai Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine (YBKA201907 and YBKA202208) and Innovative Research Team of High-Level Local Universities in Shanghai (SHSMUZDCX20210900 and SHSMU-ZDCX20210902).
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
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