Retinal Vasoproliferative Tumors Research Articles

Clinical Profiles of Retinal Vasoproliferative Tumors

Purpose: To describe the clinical features and treatment outcomes of patients with retinal vasoproliferative tumors. Methods: This retrospective case series comprised patients diagnosed with a retinal vasoproliferative tumor. Electronic medical records were reviewed, and patients’ demographic details, clinical presentation, and treatment outcomes were analyzed. Results: Nineteen eyes of 19 patients with vasoproliferative tumors were included. The mean age (±SD) at presentation was 37.0 ± 16.95 years. No eye had bilateral tumors, and 1 eye had multiple tumors. Three eyes (15%) had primary tumors, while 16 (84%) had secondary tumors. Primary tumors mainly affected the inferotemporal quadrant (n = 3). Secondary tumors involved the inferior quadrant (n = 4), inferotemporal quadrant (n = 5), and inferonasal quadrant (n = 5). Secondary tumors were associated with Coats disease (n = 6), intermediate uveitis (n = 3), traumatic chorioretinopathy (n = 2), familial exudative vitreoretinopathy (n = 2), retinal vasculitis (n = 2), and retinal vascular occlusion (n = 1). Retinochoroidal features included intraretinal and subretinal exudates, subretinal fluid, intraretinal hemorrhaging, vitreous hemorrhaging, cystoid macular edema, vitritis, preretinal fibrosis, dilated feeding vessel, epiretinal membranes, and tractional retinal detachment. Treatment modalities included cryotherapy, laser photocoagulation, and local steroids. The mean follow-up was 25.3 months, during which 18 eyes had tumor regression and 1 had a worsening condition. Conclusions: Secondary vasoproliferative tumors were more frequently observed than primary tumors, often presenting as unilateral, unifocal tumors situated posterior to the equator in the inferior fundus. Conventional treatment approaches, such as cryotherapy and laser photocoagulation, were effective at tumor regression and often required multiple sessions.

Treatment of Intermediate Uveitis-associated Retinal Vasoproliferative Tumors: A Case Series.

To report the use of combination intravitreal pharmacotherapy using anti-vascular endothelial growth factor (VEGF) and short and long-term corticosteroid implants for the treatment of retinal vasoproliferative tumors (VPT) associated with intermediate uveitis. Retrospective chart review of patients with VPT secondary to idiopathic intermediate uveitis that underwent combination intravitreal pharmacotherapy at a single center was performed. Multimodal imaging including ultrawide field color fundus photography, ultrawide field fluorescein angiography, and optical coherence tomography obtained before and after treatment were reviewed. Four eyes of 4 patients were treated with multiple injections of a combination of aflibercept, dexamethasone, and fluocinolone acetonide implants for VPT associated with intermediate uveitis. All 4 patients had improvement in visual acuity, intraocular inflammation, central macular thickness, and retinal vascular leakage, as well as regression of the VPT and reduction in lesion leakage on follow-up. Combination intravitreal anti-VEGF and corticosteroid implants may be a useful approach for the treatment of intermediate uveitis-associated VPTs and can lead to both functional and structural improvement.

Neovascular glaucoma in a pediatric patient with neurofibromatosis type 1: a case report

BackgroundTo report a case of a young patient with neurofibromatosis type 1 (NF1).Methods: Here we review the treatment administered to a 7-year-old NF1 patient with neovascular glaucoma as the primary diagnosis.Case presentationA 7-year-old boy developed visual loss in the right eye associated with periocular pain and ipsilateral headache that had persisted for 1 week. The patient’s condition did not improve after treatment with topical or systemic glaucoma medications. Fundus examination of the right eye showed superotemporal retinal vasoproliferative tumors (RVPT). Near-infrared reflectance scans of the left eye’s fundus revealed bright patchy regions, scattered across the posterior pole; systemic examination showed café-au-lait spots all over the patient’s body. The patient had a clear family history. Genetic testing confirmed NF1. The right eye was treated with intravitreal ranibizumab injection, retinal lesion cryotherapy, and transscleral ciliary body photocoagulation. After treatment, RVPT scarring was observed. The patient’s intraocular pressure remained within normal limits.ConclusionsWe report a rare case of neurofibromatosis in a pediatric patient with neovascular glaucoma accompanied by RVPT. We suggest that evaluations of young patients with neovascular glaucoma should include careful attention to the overall condition of the patient and his/her parents, as well as family history. If necessary, NF1 molecular testing should be performed to avoid a missed diagnosis or misdiagnosis.

Vasoproliferative retinal tumors in children and adults

AbstractPurposeVasoproliferative tumors of the retina (VPTR) are benign lesions of unknown origin that may occur isolated (primary) or in association with other ocular conditions (secondary). They may be unilateral or bilateral and have a predilection for the peripheral inferior temporal quadrant of the retina. Retinal vasoproliferative tumors may be present at any age, although the majority are present in the third and fourth decades of life.MethodsThe authors report a personal series of cases in young children and adults either idiopathic or secondary to a pre‐existing ocular disease (retinitis pigmentosa and Nf1).ResultsClinical findings, imaging, therapeutic options and outcome are reported.ConclusionsRetinal vasoproliferative tumors may be idiopathic (74%) or secondary to a pre‐existing ocular diseases (26%). The fundus quadrant location in these tumors is curiously weighted toward the inferotemporal region. Despite this, these tumors may effect the visual activity because of the associated retinovitreal findings, such as subretinal and/or intraretinal exudation, subretinal fluid, vitreous hemorrhage, preretinal macular fibrosis and macular edema.

Differential diagnosis of retinal capillary hemangiomas and vasoproliferative tumors

Purpose. To propose the major differential diagnostic criteria of retinal capillary hemangioma (RCH) and vasoproliferative tumor (VPT) aimed at increasing the efficiency of the diagnosis of these diseases.Material and methods. A comprehensive ophthalmologic examination off 19 (26 eyes) patients, aged 19 to 66 (ave. 31.89 ± 11.30 yrs), with vascular neoplasms of peripheral localization included, beside the standard techniques, fundus photoregistration, fluorescent angiography (FAG), optical coherence tomography of the retina and a combined ultrasound testing using B scanning, echographic densitometry, colour Doppler imaging (CDI), and pulse Doppler imaging.Results. Based on the analysis of the clinical picture and the results of the tests performed, the following differential diagnostic criteria were proposed: the age at which RCH and VPT developed, their localization in the eye fundus, and the value of the maximal systolic blood flow velocity in the central vein of the retina.Conclusion. Differential diagnostics of RCH and VPT requires combined approaches and should take into account, in addition to case history and the clinical picture, also the results of instrumental testing techniques: FAG, echography and CDI.

Comparative Study on the Efficacy and Safety of Tumor Resection in Vitrectomy for Retinal Vasoproliferative Tumors.

Purpose To investigate the efficacy and safety of combined vitrectomy with tumor resection in the treatment of retinal vasoproliferative tumors (RVPT). Methods Retrospective study. RVPT patients who underwent vitreous surgery at the Eye Hospital of Wenzhou Medical University from January 2011 to July 2017 were included. The main outcomes included treatment type, tumor activity, and best-corrected visual acuity (BCVA). Results Altogether, 16 patients with 17 eyes were enrolled with follow-up of no less than 6 months. Eight eyes were in the resection treatment group (Group R) and 9 eyes were in the conservative treatment group (Group C). Female (69%) were more common. The mean age was 50 (49.72 ± 12.92) years. Fifteen patients got unilateral onset and only one patient suffered bilaterally. The common symptoms were decreased visual acuity, floaters, and visual distortion. The preoperative BCVA ranged from hand movement to 20/20, with an average of 0.82 ± 0.75 LogMAR. Patients were all not high myopia, with a mean axial length of 23.27 ± 0.27 mm (21.61 mm to 24.67 mm). Of the retinal diseases, the epiretinal membrane was the most common, followed by vitreous hemorrhage, uveitis, subretinal fluid, and so on. Compared with the baseline BCVA, it improved more at postoperative 6 months and the last visit in Group R than in Group C (P=0.006 and P=0.033). The BCVA-improved 0.2 LogMAR or above in 6 months was 2 eyes in Group C and 7 eyes in Group R. All tumors in Group R were completely resected, whereas three in Group C (33.3%) had definite activity (P=0.008). In all samples, tumors were located on the inner side of the retina and had small vessel wall thickening and hyaline degeneration. The degree of astrocyte proliferation varies widely among different tumors. Conclusions RVPT was more likely to occur in nonhigh myopia patients. Epiretinal membrane and vitreous hemorrhage were the main causes for vitreous surgery in RVPT patients. Compared with conservative treatment, surgical resection of the tumor is more beneficial to patients on visual acuity recovery and preventing tumor relapse. It is a safe and effective way to treat RVPT.

Episcleral brachytherapy as an effective alternative in vasoproliferative tumors.

The aim of this study is to assess the results of episcleral brachytherapy as treatment of retinal vasoproliferative tumors (RVTs) in a referral Intraocular Tumors Unit (ITU). A retrospective review of all patients diagnosed with vasoproliferative tumors of the retina and treated with episcleral brachytherapy in the ITU, University Hospital of Valladolid between 2009 and 2015 was done. Five patients accomplished the inclusion criteria. All of them presented associated exudation and secondary retinal detachments (RD). Four patients had received prior treatments. Decreased tumor size and exudation regression was found in all cases after treatment. Visual acuity remained stable or increased in all patients. No recurrences have been found after twelve-months follow up. The results of the present study suggest that episcleral brachytherapy is an efficient and safe option in the management of vasoproliferative tumors, especially when large tumor or extensive subretinal fluid is present. In these cases episcleral brachytherapy could be considered as a first line treatment.

Complications in Intermediate Uveitis: Prevalence, Time of Onset, and Effects on Vision in Short-Term and Long-Term Follow-Up

ABSTRACTPurpose: To study the prevalence and time of onset of ocular complications in intermediate uveitis (IU) and to assess their effects on visual outcome in short-term and long-term follow-up.Methods: A retrospective cohort study of patients with IU who had a minimal follow-up of one year.Results: 96 patients (174 eyes, 70% females) were included with a mean age at presentation of 30 years. Children constituted 38% of all patients. Mean follow- up was 64.9 months. Pars planitis was the most common form followed by sarcoidosis and multiple sclerosis. Cystoid macular edema (CME), cataract, epiretinal membrane and posterior synechiae were the most prevalent complications. Posterior synechiae, band keratopathy, cataract and papillitis at presentation were associated with worse presenting visual acuity (VA). Of the late-onset complications, glaucoma/ocular hypertension (OHT) was the most significantly associated with worse long-term VA. Most sight-threatening complications (namely CME and glaucoma) were diagnosed at presentation while late complications predominantly affected the posterior segment and included among others peripheral retinal elevations, vasoproliferative tumors, and vitreous hemorrhage. Median LogMAR VA improved at short-term and long-term follow-up, from 0.2 to 0.1 (p<0.001). Subgroup analysis revealed significant VA improvement for eyes with all the different complications except for eyes with glaucoma/OHT.Conclusion: IU is a chronic low-grade uveitis that may be associated with protean early and late complications of the anterior or posterior segments or both. Optimal treatment regimens are imperative for the strict control of inflammation and proper management of complications thus allowing a favorable long-term prognosis.

Clinical characters and treatments of retinal vasoproliferative tumors

Retinal vasoproliferative tumors (VPT) are uncommon benign vascular tumors. They mostly occur in healthy patients, but may be associated with other chorioretinal diseases. Here we report four patients with VPT at a referral center from 2006 to 2015. Three patients denied any past history and one had a history of retinal detachment surgery. VPT-related complications included epiretinal membrane (ERM) (n=2), cystoids macular edema (n=1), and lamellar hole combined with dense cataract, rigid anterior capsule and vitreous opacity (n=1). Treatments for VPT and comorbidities included vitrectomy (VT) and membrane peeling with tumor resection (n=2), a combined treatment of photodynamic therapy (PDT) and intravitreal injection (IVI) of anti-vascular endothelial growth factor (anti-VEGF) (n=2). Tumor shrinkage was achieved in both patients treated with PDT and IVI of anti-VEGF injection. The other two patients with ERM were successfully treated with VT and tumor resection. Visual acuity improved at least two lines in three patients, and one patient had decreased vision due to cataract formation after VT. Pathology of the resected tumor in one case revealed massive gliosis with positive stain of vascular endothelial cells and glial fibrillary acidic protein stain. Yet the peeled membrane was acellular. Possible beneficial treatments for VPT and comorbidities include PDT combined with IVI of anti-VEGF, or VT and membrane peeling with tumor resection.

Verteporfin Photodynamic Therapy for the Treatment of Retinal Vasoproliferative Tumors

Verteporfin Photodynamic Therapy for the Treatment of Retinal Vasoproliferative Tumors

CRYOTHERAPY-INDUCED RELEASE OF EPIRETINAL MEMBRANE ASSOCIATED WITH RETINAL VASOPROLIFERATIVE TUMOR

To evaluate epiretinal membrane (ERM) response after cryotherapy for retinal vasoproliferative tumors (VPTs). Retrospective interventional case series. Of 16 eyes with VPT and ERM, the tumor was classified as primary in 12 (75%) eyes or secondary in 4 (25%) eyes. The median patient age was 44 years (mean, 43 years; range, 9-70 years). The tumor was located in extramacular zone (n = 16, 100%) and inferotemporal quadrant (n = 12, 75%). The mean tumor base was 6 mm, and thickness was 3 mm. The ERM involved the macula in 12 (75%) eyes and extramacular zone in 4 (25%) eyes, with best-corrected visual acuity of 20/40 or better in 6 (38%) eyes. Associated features included cystoid macular edema (n = 8, 50%), subretinal fluid (n = 10, 63%), vitreous cells (n = 9, 56%), and vitreous hemorrhage (n = 7, 44%). Single-session cryotherapy (double freeze-thaw) to the VPT was performed in each case. Over mean follow-up of 68 months (median, 54 months; range, 8-252 months), tumor regression was documented in 16 (100%) cases, with ERM release in 10 (63%) cases. After ERM release, the foveal anatomy was normal in 12 (75%) eyes. Final visual acuity improved (n = 5, 31%), remained stable (n = 9, 56%), or worsened (n = 2, 13%). Posttreatment best-corrected visual acuity was 20/40 or better in 10 (63%) eyes. Cryotherapy is remarkably effective for VPT of 6 mm or less in basal dimension. After cryotherapy, VPT-related ERM spontaneously released in 63% of the cases, without the need for surgical intervention.

Management of Idiopathic Retinal Vasoproliferative Tumors by Slit-Lamp Laser or Endolaser Photocoagulation

To report the efficacy of laser photocoagulation in treating idiopathic (primary) retinal vasoproliferative tumors. Retrospective noncomparative interventional case series. Thirty eyes of 30 patients (22 women and 8 men) with retinal vasoproliferative tumors. Main outcome measures were best-corrected visual acuity (BCVA), presence of retinal hard exudates, retinal detachment (RD), cystoid macular edema (CME), and epiretinal membrane (ERM). Laser photocoagulation was performed at the slit lamp or during pars plana vitrectomy for telangiectasia visible at the retinal vasoproliferative tumors surface. Fifteen eyes were treated with slit-lamp laser. Mean follow-up was 36.5 months (range 7 months to 11 years). Mean BCVA increased from 20/40 to 20/32 at the end of the follow-up. Hard exudates and CME regressed completely in 15 of 15 and 5 of 6 eyes, respectively. Fifteen eyes underwent vitrectomy owing to the presence of ERM (10 eyes), large exudative RD (4 eyes), or vitreous hemorrhage (1 eye). Mean follow-up was 24 months (9 months to 3.5 years). Mean BCVA increased from 20/125 to 20/40 at the end of the follow-up. No vision loss occurred. The retina reattached in all cases. Hard exudates regressed completely in 14 of 15 eyes and decreased significantly in 1. Postoperative complications were rhegmatogenous RD in 2 eyes, successfully reattached after additional surgery. Selective laser photocoagulation of retinal telangiectasia at the retinal vasoproliferative tumors' surface induced the regression of retinal exudation. Slit-lamp laser photocoagulation was sufficient in half of the cases. In more severe conditions, direct endolaser photocoagulation of the telangiectasia was required.

Retinal Vasoproliferative Tumors in 6 Patients With Neurofibromatosis Type 1

Retinal vasoproliferative tumors (RVPTs) are an important ocular finding in patients with neurofibromatosis type 1 (NF1), and early detection of this association and prompt initiation of treatment may prevent vision loss and blindness in affected patients. To describe the clinical findings of RVPTs in patients with NF1 and to underscore the risk of severe vision loss in such patients. DESIGN, SETTING, AND PATIENTS We performed a retrospective medical record review of 6 patients with RVPTs and NF1 treated at the Ocular Oncology Service, Wills Eye Hospital. The demographics, clinical features, clinical course, and outcomes of the 6 patients with RVPTs were recorded. RESULTS Of 275 patients with RVPTs, 6 (2.2%) had NF1. At the time of diagnosis of RVPT, the median patient age was 12 years (range, 9-36 years). Visual acuity was variable, ranging from 6/7.5 to light perception on initial presentation to the oncology service. The RVPT was located between the equator and ora serrata in all patients. The mean basal tumor diameter was 11 mm, and the mean tumor thickness was 4 mm. Associated features included subretinal fluid (n = 6), subretinal exudation (n = 6), epiretinal membrane (n = 3), retinal hemorrhage (n = 2), vitreous hemorrhage (n = 1), retinal neovascularization (n = 1), and cystoid macular edema (n = 1). Fluorescein angiography revealed early hyperfluorescence and late staining and leakage of each RVPT. B-scan ultrasonography revealed acoustic solidity of the lesion. Initial management included cryotherapy, intravitreal injection of bevacizumab, plaque radiotherapy, and primary enucleation in 1 patient because of painful neovascular glaucoma. We found that RVPTs can develop in patients with NF1 and can cause exudative retinopathy, vitreous hemorrhage, and visual loss. Patients with NF1 should undergo periodic ophthalmic examination for detection and treatment of this tumor. It is important to recognize the occurrence of RVPT in patients with NF1 because knowledge of this association and early treatment can prevent severe loss of vision in affected patients.

Retinal Vasoproliferative Tumors

To compare the clinical features of primary vs secondary retinal vasoproliferative tumors (VPTs). Retrospective case series of 334 tumors in 295 eyes of 275 patients. Of 275 patients with VPT, 41% (n = 113) were male and 59% (n = 162) were female, with a mean age of 44 years at presentation. Primary VPT occurred in 80% (n = 219) and secondary VPT, in 20% (n = 56) of patients. Secondary VPT (n = 67) occurred in eyes with retinitis pigmentosa (n = 15, 22%), pars planitis (n = 14, 21%), Coats disease (n = 11, 16%), previous retinal detachment surgery (n = 8, 12%), idiopathic peripheral retinal vasculitis (n = 4, 6%), familial exudative vitreoretinopathy (n = 3, 4%), and others (n = 12, 18%). The mean interval between diagnosis of underlying ocular condition and secondary VPT was 160 months. Statistically significant differences (P < .05) in clinical features (primary vs secondary VPTs) included mean age at presentation (46 vs 38 years), visual symptoms (74% vs 87%), poor visual acuity worse than 20/200 (15% vs 28%), bilaterality (4% vs 20%), multifocality (5% vs 15%), postequatorial tumor location (20% vs 33%), tumor basal dimension (6 vs 7 mm), anterior chamber cells (16% vs 30%), and vitreous cells (19% vs 48%). Retinal vasoproliferative tumor can be primary (80%) or secondary (20%). Compared with primary VPT, secondary VPT is more often bilateral, multiple, and larger and occurs at an earlier age associated with poorer visual acuity.

Retinal vasoproliferative tumors in patients with neurofibromatosis: An analysis of 5 patients

Most patients with neurofibromatosis type 1 (NF1) have no major fundus manifestations. However, rare findings include multiple choroidal nevi, myelinated nerve fibers, combined hamartoma, choroidal schwannoma and choroidal melanoma. Another fundus lesion, retinal vasoproliferative tumor, (RVPT) has recently been recognized with NF1. The authors reviewed the fundus findings and management of 5 patients with NF1 who developed RVPT. The mean patient age at recognition of RVPT was 20 years (median, 14; range, 11-36). There were 3 females and 2 males. Other ocular findings of NF1 included multiple Lisch nodules in 2 and optic nerve glioma in 1. The VPT was located between the equator and ora serrata in all cases. The mean basal diameter of VPT was 10 mm, with a mean thickness of 3 mm. Associated features included retinal detachment (n = 5), epiretinal membrane (n = 3), subretinal/intraretinal exudation (n = 5), retinal hemorrhage (n = 1), and cystoid macular edema (n = 1). The primary treatment included cryotherapy (n = 1), cryotherapy with intravitreal Bevacizumab (n = 2), sub-Tenon’s triamcinolone (n = 1), plaque radiotherapy (n = 1), and enucleation (n = 1). RVPTs have been classified into primary and secondary types. Secondary RVPTs have been observed with an increasing number of pediatric fundus conditions, including intermediate uveitis, retinitis pigmentosa, Coats’ disease, retinopathy of prematurity, familial exudative vitreoretinopathy, and others. RVPT should be added to the list of conditions that can occur with NF1 and children with NF1 should be monitored for RVPTs. This tumor can lead to severe visual loss may require vigorous treatment.

Retinal vasoproliferative tumors in ocular conditions of childhood

Retinal vasoproliferative tumor (RVPT) is a vascular mass with exudative retinopathy and minimally dilated feeder vessels. This type of tumor can occur as a primary or secondary condition. Our purpose was to describe secondary vasoproliferative tumors from ocular conditions in childhood. The clinical records and fundus photographs of patients with secondary RVPTs were reviewed and a detailed literature search was conducted to identify reported cases of secondary RVPTs. This information was used to compile a list of ocular and nonocular childhood conditions associated with development of RVPTs. The ocular conditions related to vasoproliferative tumors in children include intermediate uveitis, retinitis pigmentosa, ocular toxocariasis, Coats disease, neurofibromatosis, retinal toxoplasmosis, retinopathy of prematurity, familial exudative vitreoretinopathy, and other rare conditions. Several pediatric ocular conditions can ultimately lead to retinal vasoproliferative tumor.

The expanding spectrum of retinal vasoproliferative tumors in children

The expanding spectrum of retinal vasoproliferative tumors in children

Pars Plana Vitrectomy and Endoresection of a Retinal Vasoproliferative Tumor

A healthy 31-year-old woman developed decreased vision due to vitreous hemorrhage from 2 retinal vascular lesions. Because of the patient's desire for visual rehabilitation and the concern for von Hippel-Lindau (VHL) disease-associated retinal hemangioblastomas, pars plana vitrectomy and endoresection of both lesions were performed. Surgical techniques included the use of chandelier illumination to enable bimanual manipulation of tissue, endolaser around the tumor prior to resection, endodiathermy to cauterize the tumor's feeder vessels, and long-acting gas tamponade following the retinectomy. Histopathology showed a vascular anomaly within the retina that consisted of multiple large vessels with multiple lumens and no evidence of VHL-associated retinal hemangioblastoma. Long-term follow-up revealed no evidence of recurrent disease. This surgical technique may be valuable in select patients to differentiate between retinal vasoproliferative tumors and VHL disease-associated retinal hemangioblastomas for diagnostic, therapeutic, and counseling purposes.

Bilateral vasoproliferative retinal tumors in a patient with autosomal dominant aniridia.

To document retinal vasoproliferative tumors in a patient with aniridia. A 32-year-old woman with known autosomal dominant aniridia and lifelong visual acuity of approximately 20/80 noted further visual acuity loss. Examination revealed horizontal nystagmus and visual acuity of 20/100 in both eyes. Both eyes displayed a narrow rim of rudimentary iris with visualization of the entire lens and zonule. The cornea was clear and minimal posterior subcapsular cataract was noted. Both eyes showed diffuse retinal pigment epithelial alterations. The fovea was flat and without depression on optical coherence tomography. Inferiorly in both eyes was exudative retinal detachment from ill-defined vascular retinal tumors of approximately 15 mm diameter and 3.5 mm thickness. Both eyes were treated with plaque radiotherapy. Although aniridia typically affects the anterior segment of the eye, vision-threatening retinal detachment from vasoproliferative tumor can occur.