Chang Ying, Wei Wen Bin, Shi Ji Tong, Xian Jun Fang, Yang Wen Li, Xu Xiao Lin, Bai Hai Xia, Li Bin, Jonas Jost B
Beijing Institute of Ophthalmology, Beijing Tongren Hospital, Capital Medical University, Beijing Ophthalmology & Visual Sciences Key Laboratory, Beijing, China.
Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing Ophthalmology & Visual Sciences Key Laboratory, Beijing, China.
Acta Ophthalmol. 2016 Nov;94(7):e637-e643. doi: 10.1111/aos.13029. Epub 2016 Apr 29.
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.
The retrospective hospital-based case series study included all patients who were consecutively operated for CPE adenomas.
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.
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.
睫状体色素上皮（CPE）腺瘤是罕见的良性肿瘤，主要需与睫状体恶性黑色素瘤相鉴别。在此，我们报告一系列连续的CPE腺瘤患者，并描述其特征。
这项基于医院的回顾性病例系列研究纳入了所有因CPE腺瘤而连续接受手术的患者。
在110例接受睫状体肿瘤治疗的患者中，5例（4.5%）患有CPE腺瘤。平均年龄为59.0±9.9岁（范围：46 - 72岁）。肿瘤顶端平均厚度为6.6±1.7毫米。肿瘤颜色大多均匀为棕色至黑色，肿瘤表面光滑。肿瘤团块将虹膜组织向前推，未浸润虹膜或前房角。超声检查显示回声图不规则，病变边界清晰，彩色多普勒血流成像有血流信号。超声生物显微镜检查显示内部反射率为中低水平且有声衰减。在磁共振成像（MRI）中，与脑相比，肿瘤在T1加权图像上呈高信号，在T2加权图像上呈低信号。肿瘤组织由色素上皮细胞索和巢组成，被血管化纤维结缔组织间隔分开，呈现假腺泡样外观。细胞质中的黑色素颗粒大且大多呈球形。4例患者的肿瘤色素沉着过度。肿瘤细胞大，核呈圆形或椭圆形，核仁清晰可见。
CPE腺瘤的这些临床特征，如均匀的深棕色、表面光滑、虹膜移位和前房角变窄但无虹膜浸润、节段性白内障、色素播散，以及在T2加权MRI图像上与脑组织相比呈低信号，与眼外肌或泪腺相比呈高信号，可能有助于与睫状体恶性黑色素瘤相鉴别。
