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The phenotype of the lesion showed overlap with a vasoproliferative tumor or an astrocytic hamartoma. Over a period of 5 years of follow-up, the peripheral tumor was unchanged. No significant progression of the peripheral retinal degeneration was evidenced by autofluorescent imaging over this time period although the central acuity continued to decrease. CRB1-associated RP may be characterized by preservation of para-arteriolar retinal pigment epithelium and slow progression, and may also feature a benign peripheral retinal tumor. CRB1-a