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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 retinal hemangioblastoma. (A) The retinal hemangioblastoma was composed of classical foamy (tumor) cells admixed with small capillaries. Osseous tissues (asterisks) are adjacent to the retinal hemangioblastoma. (B) The hemangioblastoma shows high expression of vascular endothelial growth factor (VEGF). (C) Hypoxia-inducible transcription factor 1α (HIF-1α) is expressed in the retinal hemangioblastoma. (A, hematoxylin and eosin, original magnification, ×200; B and C, avidin-biotin complex immunohistochemistry, original magnification, ×200).
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Fig3: Photomicrographs of retinal hemangioblastoma. (A) The retinal hemangioblastoma was composed of classical foamy (tumor) cells admixed with small capillaries. Osseous tissues (asterisks) are adjacent to the retinal hemangioblastoma. (B) The hemangioblastoma shows high expression of vascular endothelial growth factor (VEGF). (C) Hypoxia-inducible transcription factor 1α (HIF-1α) is expressed in the retinal hemangioblastoma. (A, hematoxylin and eosin, original magnification, ×200; B and C, avidin-biotin complex immunohistochemistry, original magnification, ×200).

Mentions: A large retinal hemangioblastoma was noted at the optic nerve head (Figure 3A). The tumor was mainly composed of large cells with foamy cytoplasm (Figure 3A). Within the retinal hemangioblastoma, there were focal areas of cystic degeneration, gliosis, and hemorrhage. There was osseous transformation beneath the hemangioblastoma, mixed with gliofibrous tissues in some areas (Figure 3A). Expression of VEGF and HIF-1α was strongly positive within the hemangioblastoma and in the optic nerve (Figure 3B,C). Large hemorrhages beneath the choroid were observed.Figure 3


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 retinal hemangioblastoma. (A) The retinal hemangioblastoma was composed of classical foamy (tumor) cells admixed with small capillaries. Osseous tissues (asterisks) are adjacent to the retinal hemangioblastoma. (B) The hemangioblastoma shows high expression of vascular endothelial growth factor (VEGF). (C) Hypoxia-inducible transcription factor 1α (HIF-1α) is expressed in the retinal hemangioblastoma. (A, hematoxylin and eosin, original magnification, ×200; B and 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

Fig3: Photomicrographs of retinal hemangioblastoma. (A) The retinal hemangioblastoma was composed of classical foamy (tumor) cells admixed with small capillaries. Osseous tissues (asterisks) are adjacent to the retinal hemangioblastoma. (B) The hemangioblastoma shows high expression of vascular endothelial growth factor (VEGF). (C) Hypoxia-inducible transcription factor 1α (HIF-1α) is expressed in the retinal hemangioblastoma. (A, hematoxylin and eosin, original magnification, ×200; B and C, avidin-biotin complex immunohistochemistry, original magnification, ×200).
Mentions: A large retinal hemangioblastoma was noted at the optic nerve head (Figure 3A). The tumor was mainly composed of large cells with foamy cytoplasm (Figure 3A). Within the retinal hemangioblastoma, there were focal areas of cystic degeneration, gliosis, and hemorrhage. There was osseous transformation beneath the hemangioblastoma, mixed with gliofibrous tissues in some areas (Figure 3A). Expression of VEGF and HIF-1α was strongly positive within the hemangioblastoma and in the optic nerve (Figure 3B,C). Large hemorrhages beneath the choroid were observed.Figure 3

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