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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

Photomicrographs of neovascularization in the cornea and iris. (A) The corneal epithelium shows epidermalization. Many small vascular lumina (arrows) are located in the corneal subepithelial region. (B) Small vascular lumina (arrows) are present superficially and in the surface of the iris. (C) The anterior cornea shows high expression of vascular endothelial growth factor (VEGF). (A and B, hematoxylin and eosin, original magnification, ×200; C, avidin-biotin complex immunohistochemistry, original magnification, ×200).
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Fig2: Photomicrographs of neovascularization in the cornea and iris. (A) The corneal epithelium shows epidermalization. Many small vascular lumina (arrows) are located in the corneal subepithelial region. (B) Small vascular lumina (arrows) are present superficially and in the surface of the iris. (C) The anterior cornea shows high expression of vascular endothelial growth factor (VEGF). (A and B, hematoxylin and eosin, original magnification, ×200; C, avidin-biotin complex immunohistochemistry, original magnification, ×200).

Mentions: The enucleated eye was sent to the National Eye Institute for pathological examination. Routine histopathology and immunohistochemistry were performed on the enucleated right globe. Macroscopically, the cornea was perforated by an expulsive hemorrhage. The anterior chamber was completely occluded by a pupillary membrane admixed with intraocular contents and extensive hemorrhaging. The vitreous cavity was filled with hemorrhage, and the retina was poorly identified. There was bone tissue admixed with hemorrhages in the posterior pole. The optic nerve also contained hemorrhage. On microscopy, the cornea was perforated centrally, where the hemorrhage was mixed with the exposed intraocular contents including the uvea and retina. Most of the remaining corneal epithelium showed changes in epidermalization, and there was extensive neovascularization with small hemorrhages at the anterior corneal surface (Figure 2A). Consequently, immunostaining for VEGF was positive at the corneal surface (Figure 2C). The atrophic iris was disorganized and adhered to the Descemet’s membrane. Additionally, the surface of the iris showed neovascularization (Figure 2B). The retina was totally detached, disorganized, and showed marked gliosis.Figure 2


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)

Photomicrographs of neovascularization in the cornea and iris. (A) The corneal epithelium shows epidermalization. Many small vascular lumina (arrows) are located in the corneal subepithelial region. (B) Small vascular lumina (arrows) are present superficially and in the surface of the iris. (C) The anterior cornea shows high expression of vascular endothelial growth factor (VEGF). (A and B, hematoxylin and eosin, original magnification, ×200; C, avidin-biotin complex immunohistochemistry, original magnification, ×200).
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Fig2: Photomicrographs of neovascularization in the cornea and iris. (A) The corneal epithelium shows epidermalization. Many small vascular lumina (arrows) are located in the corneal subepithelial region. (B) Small vascular lumina (arrows) are present superficially and in the surface of the iris. (C) The anterior cornea shows high expression of vascular endothelial growth factor (VEGF). (A and B, hematoxylin and eosin, original magnification, ×200; C, avidin-biotin complex immunohistochemistry, original magnification, ×200).
Mentions: The enucleated eye was sent to the National Eye Institute for pathological examination. Routine histopathology and immunohistochemistry were performed on the enucleated right globe. Macroscopically, the cornea was perforated by an expulsive hemorrhage. The anterior chamber was completely occluded by a pupillary membrane admixed with intraocular contents and extensive hemorrhaging. The vitreous cavity was filled with hemorrhage, and the retina was poorly identified. There was bone tissue admixed with hemorrhages in the posterior pole. The optic nerve also contained hemorrhage. On microscopy, the cornea was perforated centrally, where the hemorrhage was mixed with the exposed intraocular contents including the uvea and retina. Most of the remaining corneal epithelium showed changes in epidermalization, and there was extensive neovascularization with small hemorrhages at the anterior corneal surface (Figure 2A). Consequently, immunostaining for VEGF was positive at the corneal surface (Figure 2C). The atrophic iris was disorganized and adhered to the Descemet’s membrane. Additionally, the surface of the iris showed neovascularization (Figure 2B). The retina was totally detached, disorganized, and showed marked gliosis.Figure 2

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