Presumed solitary circumscribed retinal astrocytic proliferation: the 2010 Jonathan W. Wirtschafter Lecture.

OBJECTIVE To report the clinical features and differential diagnosis of an unusual entity termed presumed solitary circumscribed retinal astrocytic proliferation (PSCRAP). METHODS Retrospective review of medical records. RESULTS All patients with PSCRAP were asymptomatic, and the lesion was found during routine examination. There were 5 men and 2 women with a median age of 53 years. No patient had a history or clinical findings of tuberous sclerosis complex. Each PSCRAP lesion was circumscribed, abruptly elevated, and opaque white to yellow and mostly obscured the underlying retinal vessels. The lesions had no associated subretinal fluid, hemorrhage, calcification, or retinal traction. Fluorescein angiography disclosed mild hyperfluorescence in the venous phase and moderate late staining of the lesions. Autofluorescence showed mild hyperautofluorescence of the lesions. Ultrasonography revealed no calcification. Optical coherence tomography showed an abruptly elevated retinal mass with optical shadowing posterior to the lesion. Six lesions were stable after a median follow-up of 6 years, and 1 lesion gradually disappeared. The pathogenesis and pathologic features of PSCRAP are unknown. CONCLUSION Presumed solitary circumscribed retinal astrocytic proliferation appears to be a unique retinal lesion of adulthood that resembles astrocytic hamartoma or retinoblastoma but displays distinctive ophthalmoscopic features.