Retinal pigment epithelial tumors are both congenital, and acquired. The most common are created by laser,, cryotherapy, infection, inflammation, trauma, neovascularization,, and long-standing SRF. However, there exist, other forms, such as congenital hypertrophy of the RPE, (CHRPE), acquired hamartomas, as well as adenomas, and adenocarcinoma (Mind map 31-1). It is important, for the eye cancer specialist to be familiar with retinal, pigment epithelial tumors, as they need to be differentiated, from choroidal melanoma.1, CHRPE, or “bear tracks”, are typically discovered in children, and young adults as multifocal, flat, inactive, and, grouped pigmented fundus lesions. They are associated, with familial adenomatous polyposis (FAP), suggesting, their treatment must be coordinated with a gastroenterologist, (see Chapter 10).2,3 Hamartomas of the RPE can, be subdivided into simple acquired hypertrophic or idiopathic, RPE hamartomas, to more complex combined, hamartomas of the retina and RPE, and unilateral RPE, dysgenesis.4,5, Retinal pigment epithelial dysplasias include idiopathic, RPE hyperplasia, adenoma, and adenocarcinoma, and, are described later in this chapter. These 3 entities are, clinically and histopathologically challenging to distinguish, and likely represent stages along a continuous, spectrum (Mind map 31-1).6
