A 52-year-old woman presents with floaters and mild metamorphopsia in the right eye for six weeks. Dilated fundus examination reveals a yellow-pink, globular, elevated retinal mass measuring approximately 2 disc diameters in the inferotemporal periphery. The structure marked **A** in the diagram is surrounded by lipid exudation extending toward the macula and overlying vitreous hemorrhage. Fluorescein angiography shows minimal feeder vessel dilatation and intrinsic hyperfluorescence with late leakage. Which of the following is the most likely diagnosis?
A. Retinal capillary hemangioblastoma associated with von Hippel–Lindau syndrome
B. Vasoproliferative tumor of the retina (primary, benign glio-vascular proliferation)
C. Retinal astrocytoma with secondary exudation
D. Retinal pigment epithelium adenoma with lipid accumulation
Explanation
Why Vasoproliferative tumor of the retina (primary, benign glio-vascular proliferation) is right
The clinical presentation—a yellow-pink, globular, elevated retinal mass in a 52-year-old woman with minimal feeder vessel dilatation, intrinsic hyperfluorescence, and late leakage on FA, surrounded by lipid exudation and vitreous hemorrhage—is pathognomonic for a primary vasoproliferative tumor of the retina (VPTR). VPTR is a benign reactive glio-vascular proliferation that accounts for 80% of cases and typically presents in middle-aged women with unilateral involvement. The structure marked A (peripheral vasoproliferative nodule) is the hallmark lesion. The minimal feeder vessel dilatation distinguishes VPTR from retinal capillary hemangioblastoma, which shows prominent dilated feeder arteries and draining veins (Shields JA, Shields CL. Intraocular Tumors. 3rd ed.).
Why each distractor is wrong
Retinal capillary hemangioblastoma associated with von Hippel–Lindau syndrome: VHL hemangioblastomas present in younger patients (<30 years), are orange-red in color, and have prominent dilated feeder artery and draining vein—features absent in this case. The minimal feeder vessel dilatation and age of presentation (52 years) exclude this diagnosis.
Retinal astrocytoma with secondary exudation: Retinal astrocytomas are rare, typically present as grayish nodules with minimal exudation, and lack the characteristic yellow-pink color and intrinsic hyperfluorescence pattern seen in VPTR.
Retinal pigment epithelium adenoma with lipid accumulation: RPE adenomas are flat, pigmented lesions without the globular elevation, intrinsic hyperfluorescence, or associated vitreous hemorrhage characteristic of the structure marked A.
