From the Ocular Oncology Service (PMK, EATS, CLS, JAS) and the Retina Service (CDR), Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania.
Support provided by the Eye Tumor Research Foundation, Philadelphia, Pennsylvania (CLS). The funders had no role in the design and conduct of the study, in the collection, analysis, and interpretation of the data, or in the preparation, review or approval of the manuscript.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Carol L. Shields, MD, Ocular Oncology Service, Suite 1440, Wills Eye Institute, 840 Walnut Street, Philadelphia, PA 19107. E-mail: firstname.lastname@example.org
Vasoproliferative tumor (VPT) of the retina is a benign vascular lesion that can occur as a unilateral or bilateral condition, often in the fundus periphery, and simulates a variety of intraocular tumors, including the more ominous retinal hemangioblastoma.1 In 1983, Shields et al. reported 12 cases of this tumor under the rubric “presumed acquired retinal hemangiomas.”1 In 1995, Shields et al. reported 103 cases and classified them as either primary (idiopathic) or secondary types.2 In that series, primary VPTs represented 76% of all cases and were seen mostly in older, hypertensive patients, whereas secondary VPTs were associated with intermediate uveitis, retinitis pigmentosa, toxoplasmic retinitis, toxocariasis, and several other conditions.1,2 To the best of our knowledge, secondary VPT has not been previously described in association with retinopathy of prematurity (ROP). We describe an advanced case of exudative VPT with total retinal detachment in an eye with a history of mild, stable ROP.
A 15-year-old girl with a history of mild ROP manifesting as mild temporal retinal dragging noted progressive blurred vision in the left eye over 7 months. Visual acuity was 20/20 in the right eye and hand motions in the left eye. Intraocular pressures and slit-lamp examination were normal in both eyes. Fundus examination of the right eye showed mild ROP-related temporal retinal dragging, with similar but much less severe features in the left eye. The left eye displayed moderate anterior vitreous inflammatory cells with premacular vitreous fibrosis. There was total shallow exudative retinal detachment with intraretinal and subretinal exudation emanating from a vascular retinal mass of 10 × 10 × 3.5 mm. The mass displayed ill-defined margins, minimally dilated feeding retinal artery and draining vein, and intraretinal hemorrhage (Fig. A). The clinical features were consistent with a VPT (secondary type) with exudative retinal detachment.
Figure. A 15-Year-Old Girl with Cicatricial Retinopathy of Prematurity in Her Left Eye Presented with Blurred Vision in Her Left Eye for 7 Months. (A) Montage Photograph of the Left Eye Showed Mild Vitritis, Vitreous Fibrosis over the Fovea, Diffuse Macular and Extramacular Exudation More in the Temporal Periphery with Total Retinal Detachment, Dragging of the Temporal Arcades, and a Vasoproliferative Tumor in the 3-o’clock Periphery. (B) Eight Months After Plaque Radiation, Tumor Regression and Significant Improvement in the Amount of Exudation and Subretinal Fluid Was Seen with Vitreomacular Traction from a Dense Epiretinal Membrane and Vitreous Fibrosis over the Macula that Needed Surgery Confirmed with Optical Coherence Tomography (inset). (C) Twenty Months After Plaque Radiation, There Was Complete Tumor Regression and Resolution of Subretinal Fluid and Exudation (inset).
Treatment options included anti-vascular endothelial growth factors, triamcinolone injection, laser photocoagulation, thermoablation, cryotherapy, or plaque radiation. Based on the large tumor size, extensive exudation, and total retinal detachment, iodine-125 plaque radiotherapy (40 Gy) was performed. The tumor reduced in thickness to 3.0 mm over 4 months. The retinal detachment resolved 8 months after treatment (Fig. B). Epiretinal membrane and significant, secondary macular pucker were evident soon thereafter, and the membrane was removed by pars plana vitrectomy. At the 20-month follow-up, visual acuity was counting fingers and tumor thickness was 1.9 mm with flat retina and no exudation (Fig. C).
The term “vasoproliferative tumor of the ocular fundus” was first coined by Shields et al. to refer to an acquired retinal vascular tumor previously referred to as “presumed acquired retinal hemangioma.”2 Primary VPTs are usually solitary, located in the inferotemporal aspect of the peripheral retina, and associated with exudation and subretinal fluid.2 Although most VPTs are unilateral, secondary VPTs are bilateral in 41% of cases and can present atypically as a diffuse mass.2 Secondary VPT is often associated with intermediate uveitis, pars planitis, or degenerative retinal disease such as retinitis pigmentosa, and its association with ROP could feasibly be explained by the vascular endothelial growth factor and erythropoietin stimulus present during active ROP; however, it is unusual in the burnt-out cicatricial phase of ROP such as that seen in our patient.3 The occurrence of a VPT with stable ROP in our patient may indicate an alternate pathogenesis of VPT other than or in addition to vascular endothelial growth factor and erythropoietin.
Considering the natural course of VPTs, most lesions are benign and some patients show little or no vision loss after years of follow-up.4 Occasionally, VPTs can cause significant visual loss from vitreous hemorrhage, exudation, and subretinal fluid, and, rarely, eyes with VPTs require enucleation due to intense pain from neovascular glaucoma.2 Eyes with VPT can benefit from early laser photocoagulation, photodynamic therapy, thermoablation, or cryotherapy, especially when lesions are less than 3.0 mm in thickness.5,6 For advanced cases and those with a thickness greater than 5.0 mm or with a basal dimension greater than 6.0 mm, plaque radiotherapy is effective, as was seen in our patient.5,7 Although both iodine-125 and ruthenium-106 have been employed for VPTs, iodine-125 has been our preferred isotope because it has greater tumor penetration than ruthenium.5 The success rate with plaque radiation is impressive. Cohen et al. studied 30 eyes with VPT treated with plaque radiotherapy and found 97% and 91% of treated eyes showing complete regression and decreased exudative retinal detachment, respectively.5 Further, because the radiation dose is less than that used in treating malignant intraocular tumors such as uveal melanoma, complication rates are expected to be lower, and visual acuity either improves or remains stable in the majority of patients (73%).5
Considering the satisfactory anatomic response to treatment that our patient showed, final visual acuity remained relatively poor. This is in agreement with previous reports noting that patients with secondary VPTs are 19 times less likely to have visual acuity improvement after treatment than those with primary VPTs.5 In our case, the poor vision likely arose from the combined effects of chronic foveal traction from cicatricial ROP, secondary amblyopia, retinal detachment involving the macula, epiretinal membrane, and chronic macular edema.
VPTs are benign retinal vascular tumors that generally have a benign clinical course. Advanced cases with significant exudation and retinal detachment may benefit from early, aggressive treatment with plaque radiotherapy. However, functional recovery may be limited in patients with VPTs, particularly when there is retinal detachment involving the macula and other secondary macular effects, such as epiretinal membrane.
- Shields JA, Decker WL, Sanborn GE, Augsburger JJ, Goldberg RE. Presumed acquired retinal hemangiomas. Ophthalmology. 1983;90:1292–1300.
- Shields CL, Shields JA, Barrett J, De Potter P. Vasoproliferative tumors of the ocular fundus: classification and clinical manifestations in 103 patients. Arch Ophthalmol. 1995;113:615–623.
- Sato T, Kusaka S, Shimojo H, Fujikado T. Vitreous levels of erythropoietin and vascular endothelial growth factor in eyes with retinopathy of prematurity. Ophthalmology. 2009;116:1599–1603. doi:10.1016/j.ophtha.2008.12.023 [CrossRef]
- Say EAT, Shields CL. Retinal vasoproliferative tumor. In: Jaeger E, ed. The Wills Eye Five Minute Consult. Philadelphia: Lippincott Williams and Wilkins; In Press.
- Cohen VM, Shields CL, Demirci H, Shields JA. Iodine I 125 plaque radiotherapy for vasoproliferative tumors of the retina in 30 eyes. Arch Ophthalmol. 2008;126:1245–1251. doi:10.1001/archopht.126.9.1245 [CrossRef]
- Shields JA, Shields CL. Vasoproliferative tumor of the ocular fundus. In: Shields JA, Shields CL, eds. Atlas of Intraocular Tumors. Philadelphia: Lippincott Williams and Wilkins; 1999:396–398.
- Gibran SK. Trans-vitreal endoresection for vasoproliferative retinal tumours. Clin Experiment Ophthalmol. 2008;36:712–716. doi:10.1111/j.1442-9071.2008.01880.x [CrossRef]