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. 2000 Oct;84(10):1162–1169. doi: 10.1136/bjo.84.10.1162

Vasoproliferative tumours of the retina

H Heimann 1, N Bornfeld 1, O Vij 1, S Coupland 1, N Bechrakis 1, U Kellner 1, M Foerster 1
PMCID: PMC1723269  PMID: 11004104

Abstract

BACKGROUND—Vasoproliferative tumours of the retina (VPTR) are benign tumours of unknown origin, occurring mostly in otherwise healthy patients. VPTR may be associated with other chorioretinal diseases, such as uveitis. The tumours, which histologically represent reactive gliovascular proliferations, are characterised by a pink to yellow appearance on funduscopy and are accompanied by exudative and haemorrhagic changes of the retina.
METHODS—22 cases of VPTR in 21 patients were examined with a follow up period between 1 month and 6 years. Ophthalmological changes associated with VPTR were intraretinal and subretinal exudations (n=18), exudative detachments of the surrounding sensory retina (n=13), intraretinal and subretinal haemorrhages (n=10), exudative changes within the macula (n=10), hyperpigmentation of the retinal pigment epithelium at the border of the exudative retinal changes (n=9), and vitreous haemorrhages (n=4). Tumour biopsy was performed in two cases. Treatment consisted of plaque radiotherapy (n=14), plaque radiotherapy and cryotherapy (two), cryotherapy only (two), observation (three), and enucleation in one case of a blind and painful eye.
RESULTS—Regression of the tumour and the associated exudative changes could be observed in all treated cases. Visual acuity at last follow up improved two lines or more in two cases, remained within two lines of the initial visual acuity in 15 cases, and worsened in the remaining five. Histopathological examination of the biopsy specimens and the tumour of the enucleated eye showed massive capillary proliferation with perivascular spindle-shaped glial cells of retinal origin.
CONCLUSION—The correct diagnosis of VPTR is of importance as these lesions may lead to visual loss. Further, VPTR must be differentiated from angiomas associated with von Hippel-Lindau disease as well as from ocular and systemic malignancies. Regression of tumour thickness and associated retinal changes can be achieved with brachytherapy or cryotherapy.



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Figure 1  .

Figure 1  

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(A) VPTR in the lower retinal periphery in a 31 year old male patient who presented with floaters and loss of vision. The tumour (long arrow) is accompanied by an exudative retinal detachment, intraretinal accumulations of lipoid exudates, telangiectactic alterations of the retinal vessels (small arrow), and marked hyperpigmentations of the retinal pigment epithelium in the retina adjacent to the exudative detachment. (B) Symptomless VPTR in the lower retinal periphery in a 65 year old female patient. (C) Mid-phase fluorescein angiography of the tumour shown in (B) demonstrating the connection of tumour vessels with non-dilated retinal vessels. (D) Late phase fluorescein angiography of the tumour shown in (B) demonstrating preretinal leakage of dye on the tumour surface. (E) VPTR as shown in (B, C) 6 months after brachytherapy with 106-ruthenium. Regression of tumour thickness and exudative retinal changes. Chorioretinal scarring within the area that was covered by the 106-ruthenium plaque. (F) VPTR in the lower retinal periphery of a 15 year old boy with a history of toxoplasmosis retinochoroiditis. (G) Enucleation specimen with VPTR of the retinal periphery in a 27 year old male patient who presented with a blind and painful eye as a result of neovascular glaucoma. (H) Low power magnification of the retinal tumour demonstrating that it consists of a proliferation of glial cells and small blood vessels embedded in a hyalinised stromal matrix (haematoxylin and eosin stain, ×10 objective). (I) High power magnification of a hyalinised blood vessel surrounded by glial cells (haematoxylin and eosin stain, ×40 objective). (J) High power magnification of a blood vessel filled with erythrocytes and surrounded by glial cells and hyalinised stroma (Gomori stain, ×40 objective). (A and B were assembled using digitised fundus photographs and Adobe Photoshop software).

Figure 2  .

Figure 2  

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(A) VPTR of the lower nasal periphery of the right eye in a 31 year old male patient. Although the patient complained of visual loss and floaters for more than 6 months, diagnosis was delayed because no fundus examination had been performed by his ophthalmologist during this period. The tumour was biopsied following pars plana vitrectomy. (B) Massive intraretinal and subretinal exudations extending to the central retina in the same patient as (A), accompanied by retinal neovascularisations on the optic disc. (C) Same patient as in (A). Chorioretinal scar and complete regression of VPTR after 106-ruthenium brachytherapy. (D) Same patient as in (B). Complete regression of the central retinal exudates 1 year after 106-ruthenium brachytherapy. Note the residual changes of the RPE within the central macula. No significant gain in visual acuity could be noted despite regression of the exudative changes. (E) The biopsy specimen obtained from the tumour displayed in Figure 1 stained for glial fibrillary acid protein (GFAP, ×40 objective), demonstrating that the spindle cell population within the retinal tumours are of glial cell origin. The endothelium of the blood vessels is negative for this marker. (F) Left eye of the same patient as in (A). Initially, no fundus abnormalities could be noted in his fellow eye. After 1 year of follow up, a small VPTR could be seen in the lower retinal periphery of his fellow left eye. (G) Same tumour as in (F) demonstrating tumour growth after 4 weeks of follow up. The tumour was then treated with cryotherapy (picture taken with scleral indentation). (H) Fluorescein angiography of the same tumour as in (G) demonstrating the connections between the retinal vasculature and the tumour vessels.

Selected References

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