This report describes the histopathological findings of an iris melanoma that developed in a patient with retinitis pigmentosa (RP). A 66-year-old man was referred to our hospital with a diagnosis of iris tumor. He had a medical history of RP for over 25 years. His best corrected visual acuity at presentation was 20/20 (OD) and 20/200 (OS). Slit lamp examination revealed an iris melanocytoma in his right eye and a brown iris mass involving the pupil in his left eye. Ocular fundus examination of his right eye showed diffuse chorioretinal atrophy with attenuated retinal vessels and scattered pigment. A diagnosis of iris melanoma was made and enucleation of his left eyeball was performed. Histopathological examination of the eyeball showed an iris tumor with proliferation of spindle cells positive for Melan-A, HMB-45, and S-100 protein by immunohistochemistry. Diffuse destruction of retinal architecture was observed, with loss of outer segment and replacement by glial cells. Accumulation of melanin pigment around retinal vessels was found in peripheral retina. These histopathological findings were compatible with advanced stage of RP. This case demonstrates that iris melanoma can occur in eye with RP.
Ocul Oncol Pathol

中文翻译：

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色素性视网膜炎患者的虹膜黑色素瘤

本报告描述了视网膜色素变性 (RP) 患者发生的虹膜黑色素瘤的组织病理学发现。一名 66 岁男性因诊断为虹膜瘤被转诊到我院。他有超过 25 年的 RP 病史。他在就诊时的最佳矫正视力为 20/20 (OD) 和 20/200 (OS)。裂隙灯检查显示右眼有虹膜黑色素细胞瘤，左眼瞳孔有褐色虹膜肿块。右眼眼底检查显示弥漫性脉络膜视网膜萎缩，视网膜血管变薄，色素散在。诊断为虹膜黑色素瘤，并对他的左眼球进行了摘除术。眼球的组织病理学检查显示虹膜肿瘤伴有梭形细胞增殖，Melan-A、HMB-45、和 S-100 蛋白通过免疫组织化学。观察到视网膜结构的弥漫性破坏，外段丢失并被神经胶质细胞替代。在周围视网膜中发现视网膜血管周围黑色素的积累。这些组织病理学发现与 RP 的晚期相一致。该病例表明虹膜黑色素瘤可发生在患有 RP 的眼睛中。
眼肿瘤病理