Retinal Pigment Epithelial Tumors

Tumors of the retinal pigment epithelium (RPE) can be congenital or acquired. In this chapter, we review the clinical features of congenital and acquired tumors of the RPE and their possible systemic associations. In fact, those present at birth can be associated with systemic conditions such as familial adenomatous polyposis (FAP) or neurofibromatosis 2 (NF2). While most congenital tumors have quite characteristic clinical features that allow their accurate diagnosis, acquired RPE tumors are sometimes difficult to differentiate from choroidal neoplasms; therefore, it’s important to know the characteristics that RPE tumors show at the multimodal imaging.