title: Clinical and histopathological features of adenomas of the ciliary pigment epithelium.
abstract: OBJECTIVE
  Adenomas of the ciliary pigment epithelium (CPE) are rare benign tumours which have mainly to be differentiated from malignant ciliary body melanomas. Here we report on a consecutive series of patients with CPE adenomas and describe their characteristics.
  
  
  METHODS
  The retrospective hospital-based case series study included all patients who were consecutively operated for CPE adenomas.
  
  
  RESULTS
  Of the 110 patients treated for ciliary body tumours, five patients (4.5%) had a CPE adenoma. Mean age was 59.0 ± 9.9 years (range: 46-72 years). Mean tumour apical thickness was 6.6 ± 1.7 mm. Tumour colour was mostly homogenously brown to black, and the tumour surface was smooth. The tumour masses pushed the iris tissue forward without infiltrating iris or anterior chamber angle. Sonography revealed an irregular echogram with sharp lesion borders and signs of blood flow in Color Doppler flow imaging. Ultrasonographic biomicroscopy demonstrated medium-low internal reflectivity and acoustic attenuation. In magnetic resonance imaging (MRI), the tumours as compared to brain were hyperintense on T1-weighted images and hypointense on T2-weighted images. Tumour tissue consisted of cords and nests of pigment epithelium cells separated by septa of vascularized fibrous connective tissue, leading to a pseudo-glandular appearance. The melanin granules in the cytoplasm were large and mostly spherical in shape. In four patients, the tumours were hyperpigmented. Tumour cells were large with round or oval nuclei and clearly detectable nucleoli.
  
  
  CONCLUSIONS
  These clinical characteristics of CPE adenomas, such as homogenous dark brown colour, smooth surface, iris dislocation and anterior chamber angle narrowing but no iris infiltration, segmental cataract, pigment dispersion, and, as compared to brain tissue, hypointensity and, as compared to extraocular muscles or lacrimal gland, hyperintensity on T2-weighted MRI images, may be helpful for the differentiation from ciliary body malignant melanomas.
authors.affiliation: Beijing Institute of Ophthalmology, Beijing Tongren Hospital, Capital Medical University, Beijing Ophthalmology & Visual Sciences Key Laboratory, Beijing, China.
authors.forename: Ying
authors.initials: Y
authors.lastname: Chang
authors.affiliation: Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing Ophthalmology & Visual Sciences Key Laboratory, Beijing, China.
authors.forename: Wen Bin
authors.initials: WB
authors.lastname: Wei
authors.affiliation: Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing Ophthalmology & Visual Sciences Key Laboratory, Beijing, China.
authors.forename: Ji Tong
authors.initials: JT
authors.lastname: Shi
authors.affiliation: Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing Ophthalmology & Visual Sciences Key Laboratory, Beijing, China.
authors.forename: Jun Fang
authors.initials: JF
authors.lastname: Xian
authors.affiliation: Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing Ophthalmology & Visual Sciences Key Laboratory, Beijing, China.
authors.forename: Wen Li
authors.initials: WL
authors.lastname: Yang
authors.affiliation: Beijing Institute of Ophthalmology, Beijing Tongren Hospital, Capital Medical University, Beijing Ophthalmology & Visual Sciences Key Laboratory, Beijing, China.
authors.forename: Xiao Lin
authors.initials: XL
authors.lastname: Xu
authors.affiliation: Beijing Institute of Ophthalmology, Beijing Tongren Hospital, Capital Medical University, Beijing Ophthalmology & Visual Sciences Key Laboratory, Beijing, China.
authors.forename: Hai Xia
authors.initials: HX
authors.lastname: Bai
authors.affiliation: Beijing Institute of Ophthalmology, Beijing Tongren Hospital, Capital Medical University, Beijing Ophthalmology & Visual Sciences Key Laboratory, Beijing, China. libin43_99@163.com.
authors.forename: Bin
authors.initials: B
authors.lastname: Li
authors.affiliation: Department of Ophthalmology, Medical Faculty Mannheim, Ruprecht-Karls-University of Heidelberg, Seegartenklinik Heidelberg, Germany.
authors.forename: Jost B
authors.identifier: http://orcid.org/0000-0003-2972-5227
authors.initials: JB
authors.lastname: Jonas
mesh_terms: D000236:Adenoma / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D000368:Aged; D002924:Ciliary Body / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D033401:Microscopy, Acoustic; D008875:Middle Aged; D013508:Ophthalmologic Surgical Procedures; D010857:Pigment Epithelium of Eye / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D012189:Retrospective Studies; D014604:Uveal Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D014792:Visual Acuity
keywords: adenoma of the ciliary pigment epithelium; ciliary body tumour; ciliary malignant melanoma; ocular pathology