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Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 742-744

Aggressive retinal hemangioblastoma with scleral penetration in Von-Hippel-Lindau syndrome

Department of Ophthalmology, Government Medical College, Kozhikode, Kerala, India

Correspondence Address:
Dr. Ferzana Mohammed
Department of Ophthalmology, Government Medical College, Kozhikode, Kerala - 673 008
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_3585_20

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Retinal hemangioblastoma is associated with Von-Hippel–Lindau syndrome. Here, we report the case of a 50-year-old man who had a retinal hemangioblastoma that penetrated the sclera and presented as an extra-ocular mass. Computerized tomography of the abdomen showed bilateral renal cell carcinoma, renal, and pancreatic cysts. A provisional diagnosis of Von-Hippel–Lindau syndrome was made. Enucleation of the eyeball was done, and the diagnosis of retinal hemangioblastoma was confirmed by histopathology and immunohistochemistry. Though rare, retinal hemangioblastomas can present with extrascleral extension. In such cases, enucleation may be the only alternative.

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