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Pathology characteristics of ocular von Hippel-Lindau disease with neovascularization of the iris and cornea: a case report.

Chen S, Chew EY, Chan CC - J Med Case Rep (2015)

Bottom Line: The tumor was mainly composed of large cells with foamy cytoplasm.Bone formation was also present.Our pathology findings were consistent with previously described features of retinal hemangioblastoma.

View Article: PubMed Central - PubMed

Affiliation: Immunopathology Section, Laboratory of Immunology, National Eye Institute, National Institutes of Health, Bethesda, MD, USA. chenshida123@hotmail.com.

ABSTRACT

Introduction: Retinal hemangioblastoma is one of the most common tumors in von Hippel-Lindau disease. In addition to the classical pathological characteristics of von Hippel-Lindau disease, we report, for what we believe to be the first time, a severe and rare ocular complication characterized by neovascularization in the cornea and iris.

Case presentation: A 41-year-old white man with a long history of retinal hemangioblastoma presented with neovascularization of his iris and cornea as well as corneal perforation. His right eye was blind and painful, leading to a decision of enucleation. On microscopy, the enucleated eye showed neovascularization of the cornea and iris. The cornea was perforated with an expulsive hemorrhage and extruding intraocular contents, including the retina. A large retinal hemangioblastoma was located at the posterior pole adjacent to the optic nerve head. The tumor was mainly composed of large cells with foamy cytoplasm. Bone formation was also present.

Conclusion: Our pathology findings were consistent with previously described features of retinal hemangioblastoma. The present case is unusual because of the co-existing neovascularization in the iris and cornea, which may have led to corneal perforation and vision loss.

No MeSH data available.


Related in: MedlinePlus

Clinical photographs of the eye from the patient with von Hippel-Lindau disease. (A) The right eye shows neovascularization of the cornea and iris (rubeosis iridis) and matured cataract. (B) The left eye shows a hemangioblastoma involving the optic nerve.
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Fig1: Clinical photographs of the eye from the patient with von Hippel-Lindau disease. (A) The right eye shows neovascularization of the cornea and iris (rubeosis iridis) and matured cataract. (B) The left eye shows a hemangioblastoma involving the optic nerve.

Mentions: A 41-year-old white man was diagnosed with VHL with multiple retinal hemangioblastomas in 1987 at the age of 17 years. He received thermal laser and cryotherapy treatment for a retinal hemangioblastoma in his right eye in 1992. The tumor progressed and upon examination in June 2007 he had no light perception in his right eye, with a completely obscured fundus. On examination in April 2011, there was still no light perception in his right eye; his intraocular pressure was 52mmHg; and band keratopathy, rubeosis iridis, and dense cataracts were present (Figure 1A). On examination in July 2012, his right eye was blind and painful with an intraocular pressure of 48mmHg and a corneal ulcer. The vision in his left eye was 20/20, and the fundus showed evidence of a retinal hemangioblastoma involving the optic nerve and the presence of retinal exudates (Figure 1B). He underwent enucleation of the blind and painful right eye in November 2013.Figure 1


Pathology characteristics of ocular von Hippel-Lindau disease with neovascularization of the iris and cornea: a case report.

Chen S, Chew EY, Chan CC - J Med Case Rep (2015)

Clinical photographs of the eye from the patient with von Hippel-Lindau disease. (A) The right eye shows neovascularization of the cornea and iris (rubeosis iridis) and matured cataract. (B) The left eye shows a hemangioblastoma involving the optic nerve.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4389714&req=5

Fig1: Clinical photographs of the eye from the patient with von Hippel-Lindau disease. (A) The right eye shows neovascularization of the cornea and iris (rubeosis iridis) and matured cataract. (B) The left eye shows a hemangioblastoma involving the optic nerve.
Mentions: A 41-year-old white man was diagnosed with VHL with multiple retinal hemangioblastomas in 1987 at the age of 17 years. He received thermal laser and cryotherapy treatment for a retinal hemangioblastoma in his right eye in 1992. The tumor progressed and upon examination in June 2007 he had no light perception in his right eye, with a completely obscured fundus. On examination in April 2011, there was still no light perception in his right eye; his intraocular pressure was 52mmHg; and band keratopathy, rubeosis iridis, and dense cataracts were present (Figure 1A). On examination in July 2012, his right eye was blind and painful with an intraocular pressure of 48mmHg and a corneal ulcer. The vision in his left eye was 20/20, and the fundus showed evidence of a retinal hemangioblastoma involving the optic nerve and the presence of retinal exudates (Figure 1B). He underwent enucleation of the blind and painful right eye in November 2013.Figure 1

Bottom Line: The tumor was mainly composed of large cells with foamy cytoplasm.Bone formation was also present.Our pathology findings were consistent with previously described features of retinal hemangioblastoma.

View Article: PubMed Central - PubMed

Affiliation: Immunopathology Section, Laboratory of Immunology, National Eye Institute, National Institutes of Health, Bethesda, MD, USA. chenshida123@hotmail.com.

ABSTRACT

Introduction: Retinal hemangioblastoma is one of the most common tumors in von Hippel-Lindau disease. In addition to the classical pathological characteristics of von Hippel-Lindau disease, we report, for what we believe to be the first time, a severe and rare ocular complication characterized by neovascularization in the cornea and iris.

Case presentation: A 41-year-old white man with a long history of retinal hemangioblastoma presented with neovascularization of his iris and cornea as well as corneal perforation. His right eye was blind and painful, leading to a decision of enucleation. On microscopy, the enucleated eye showed neovascularization of the cornea and iris. The cornea was perforated with an expulsive hemorrhage and extruding intraocular contents, including the retina. A large retinal hemangioblastoma was located at the posterior pole adjacent to the optic nerve head. The tumor was mainly composed of large cells with foamy cytoplasm. Bone formation was also present.

Conclusion: Our pathology findings were consistent with previously described features of retinal hemangioblastoma. The present case is unusual because of the co-existing neovascularization in the iris and cornea, which may have led to corneal perforation and vision loss.

No MeSH data available.


Related in: MedlinePlus