Adenoma and adenocarcinoma of the retinal pigment epithelium (RPE) are rare intraocular tumours that are often misdiagnosed as posterior uveal melanoma or other simulating lesions. It is important to be able to differentiate these tumours from melanoma for 3 reasons. First, an inability to rule out melanoma often tilts the management towards enucleation. Second, management options like radiotherapy and local resection which work well for melanoma may not be easily applied to these tumours. Third, and most importantly, patients with melanoma need a lifetime follow-up to rule out metastases (metastatic dormancy) whereas RPE tumours hardly metastasize. An abruptly elevated, often deeply pigmented tumour, with a prominent retinal feeding artery and a draining vein causing exudation, should raise a suspicion of RPE tumours. RPE tumours have a remarkable local invasive potential but a low metastatic potential. Most RPE tumours require treatment due to local complications. Small, asymptomatic tumours can be generally observed. Enucleation is still the gold standard of treatment, although local resection has been reported in selected cases with good results. Here, we provide a comprehensive review of the demographic, clinical, and imaging features of true acquired neoplasms of the RPE, namely adenoma and adenocarcinoma, the ways to differentiate them from melanoma, their clinical course and prognosis, and Options for their management.
Ocul Oncol Pathol

中文翻译：

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视网膜色素上皮腺瘤和腺癌：综述

视网膜色素上皮 (RPE) 的腺瘤和腺癌是罕见的眼内肿瘤，经常被误诊为后葡萄膜黑色素瘤或其他模拟病变。由于三个原因，能够将这些肿瘤与黑色素瘤区分开来很重要。首先，无法排除黑色素瘤通常会使管理倾向于去核。其次，对黑色素瘤有效的放疗和局部切除等管理方案可能不容易应用于这些肿瘤。第三，也是最重要的，黑色素瘤患者需要终生随访以排除转移（转移休眠），而 RPE 肿瘤几乎不转移。突然隆起、通常色素沉着的肿瘤，伴有明显的视网膜供血动脉和引流静脉导致渗出，应怀疑 RPE 肿瘤。RPE 肿瘤具有显着的局部侵袭潜力，但转移潜力低。由于局部并发症，大多数 RPE 肿瘤需要治疗。通常可以观察到小的、无症状的肿瘤。剜除术仍然是治疗的金标准，尽管在选定的病例中报告了局部切除并取得了良好的效果。在这里，我们全面回顾了 RPE 真正获得性肿瘤（即腺瘤和腺癌）的人口统计学、临床和影像学特征、将它们与黑色素瘤区分开来的方法、它们的临床过程和预后，以及它们的管理选择。尽管已在选定的病例中报告了局部切除并取得了良好的效果。在这里，我们全面回顾了 RPE 真正获得性肿瘤（即腺瘤和腺癌）的人口统计学、临床和影像学特征、将它们与黑色素瘤区分开来的方法、它们的临床过程和预后，以及它们的管理选择。尽管已在选定的病例中报告了局部切除并取得了良好的效果。在这里，我们全面回顾了 RPE 真正获得性肿瘤（即腺瘤和腺癌）的人口统计学、临床和影像学特征、将它们与黑色素瘤区分开来的方法、它们的临床过程和预后，以及它们的管理选择。
眼肿瘤病理