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Case Reports
. 2013 Aug;48(4):e67-71.
doi: 10.1016/j.jcjo.2013.01.014.

Scleral penetration of an unusually aggressive case of a retinal hemangioblastoma

Murilo Rodrigues, Nicholas T Iliff, Charles G Eberhart, Silvia Montaner, Akrit Sodhi
Case Reports

Scleral penetration of an unusually aggressive case of a retinal hemangioblastoma

Murilo Rodrigues et al. Can J Ophthalmol. 2013 Aug.
No abstract available

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Figures

Figure 1
Figure 1
External photos of the highly vascularized lesion. A. Dilated tortuous vessels involving the nasal, superior, and temporal conjunctiva feed a highly vascularized subconjunctival lesion. The cornea is translucent; there is florid neovascularization with a layered hyphema in the anterior chamber. B. Views of the superior conjunctiva reveal that the lesion overrides the corneal limbus superiorly.
Figure 2
Figure 2
Ultrasound exam of the vascularized lesion. A. and B. Transverse (cross-section; A) and longitudinal (radial section; B) images using a 20 MHz probe (using a fluid standoff) of the anterior segment reveals that the lesion (L) on the outer surface of the globe is highly reflective with low reflective areas consistent with vessels and/or cystic spaces (arrow); the lesion protrudes through the sclera (arrow heads). C. Contact B-scan (10 MHz) showing the disorganized intraocular structures (V) and the marked calcification of the globe wall (asterick) with the associated acoustic shadowing (S).
Figure 3
Figure 3
Microscopic examination. A. and B. The highly vascularized intraocular lesion extended through a defect in the superior limbus (arrows) and formed an extraocular mass (asterisk, original magnifications 20X and 40X). The lesion was covered by conjunctival epithelium. C. and D. The tumor largely replaced the retina and choroid, and bony RPE metaplasia was noted in the posterior portion of the globe (asterisks, original magnifications 20X and 40X). E and F. The hemangioblastoma was composed of thin-walled, capillary-like blood vessels (arrows) and numerous lipidized stromal (tumor) cells (original magnification 400X).
Figure 4
Figure 4
Immunohistochemical analysis. The lesion contained numerous small vessels that stained positive for the endothelial marker CD34. The lipidized stromal (tumor) cells had a low proliferation index, as demonstrated by only rare Ki67 positive cells. There were entrapped glial elements (GFAP) and rare macrophages (CD68). The tumor cells were immunonegative for EMA and CAM5.2. (Original magnifications 200X or 400X).
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