睫状体肿瘤的临床病理分析

Clinical pathology of ciliary body tumors:A retrospective study of 17 cases

目的分析、总结睫状体肿瘤的临床表现和病理特征,探讨和研究其鉴别要点。方法回顾性分析我院十年来收治的17例睫状体肿瘤的临床、影像和病理资料。结果17例睫状体肿瘤中恶性黑色素瘤10例、腺瘤2例、髓上皮瘤1例(低度恶性)、平滑肌瘤1例、良性神经鞘瘤1例、黑色素细胞瘤1例、黑色素细胞瘤恶变1例。良、恶性比例为5:12。2例腺瘤因其独特的临床表现,术前基本确诊,行局部切除;平滑肌瘤、神经鞘瘤、黑色素细胞瘤因瘤体小于3个钟点、厚度小于4mm也行肿块局部切除。5例均保存了有用的视功能。黑色素细胞瘤恶变的患者有青光眼手术史。本组病例中恶性黑色素瘤均接近或大于1个象限,多数直径在10mm以上,后缘均达到锯齿缘,病理表现为上皮样细胞型。3例继发青光眼,其中2例肿瘤大片坏死,产生严重的眼内炎症状。9例为棕黑色实质性占位,1例呈黄白色(无色素型),球表常有哨兵血管,可有玻璃体种植或巩膜侵犯。在本组睫状体肿瘤的诊断中B超有一定的鉴别价值。核磁共振MRI常能发现睫状体占位,并能提示巩膜是否被累及。超声生物显微镜(ultra-sound biomicroscope,UBM)有很强的定位价值。结论睫状体肿瘤的鉴别诊断主要有赖于临床体征。基底在1个象限以上、直径达10mm的实质性占位、继发青光眼、巩膜有色素侵蚀、MRI巩膜影模糊等均提示恶性肿瘤,尤以黑色素瘤可能性大。2个钟点以内、厚度4mm以下的安静睫状体占位要考虑良性肿瘤可能。

Objective To describe the characteristics of ciliary body tumors and try to identify the key differences. Methods Seventeen eases of ciliary body tumors treated in our hospital during the last ten years were analyzed, focusing on the clinical, histopathological and imaging findings. Results Seventeen cases of ciliary body tumors were classified. They included melanoma （ten cases）, adenoma （two cases）, medulloepithelioma （one case）, leiomyoma （one case）, neurilemoma （one case）, and melanocytoma （two cases, one with malignant transformation）. The benign/malignant ratio was 5/12. All five benign tumors were managed by lamellar sclerouvectomy due to their small size （less than three clocks, thinner than 4mm thickness）. All ten cases of melanoma were no less than one quarter with the diameter of the bases more than 10mm and the posterior borders beyond the ora serrata. Vitreous seeding, sentinel vessel, and chamber angle invasion were common findings. All eyes with malignant tumors were enucleated. Ultrasound biomicroscopy （UBM） was especially valuable in the diagnosis and measurement of ciliary body tumors. Scleral invasion was found with MRI. Conclusion Most of the diagnoses of different ciliary body tumors can be made by clinical and imaging findings. A tumor with more than a 10 mm base, secondary glaucoma, or scleral invasion may indicate malignancy, especially melanoma. Small, ＂quiet＂ tumors with a thickness less than 4mm may be benign lesions.