Abstract
AbstractPurpose Adenocarcinomas of the retinal pigment epithelium (RPE) are rare adult tumors, treated with enucleation or surgical excision. We present a child with an adenocarcinoma of RPE, treated with protontherapy.Methods A 10‐year‐old girl with loss of vision had a pigmented macular tumor, diagnosed as hamartoma. Five years later, tumor growth led to a transvitreal incisional biopsy.Results Histopathology and immunohistochemistry were compatible with an adenocarcinoma of the RPE. The tumor was irradiated. After 5 years of follow‐up, the tumor is under control and the patient presents no distant metastases.Conclusion Adenocarcinomas of the RPE can simulate a combined hamartoma of the retina and RPE. Diagnosis can only be established after biopsy. Protontherapy is a valid therapeutic alternative.
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