title: Retinal vasoproliferative tumors in 6 patients with neurofibromatosis type 1.
abstract: OBJECTIVE
  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.
  
  
  OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: The Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: Jerry A
authors.initials: JA
authors.lastname: Shields
authors.affiliation: The Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: Marco
authors.initials: M
authors.lastname: Pellegrini
authors.affiliation: The Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania2The Ocular Oncology Service, L. V. Prasad Eye Institute, Hyderabad, India.
authors.forename: Swathi
authors.initials: S
authors.lastname: Kaliki
authors.affiliation: The Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: Arman
authors.initials: A
authors.lastname: Mashayekhi
authors.affiliation: The Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: Carol L
authors.initials: CL
authors.lastname: Shields
mesh_terms: D000293:Adolescent; D000328:Adult; D001766:Blindness / Q000209:etiology; D002199:Capillary Permeability; D002648:Child; D005260:Female; D019793:Fluorescein / Q000378:metabolism; D005451:Fluorescein Angiography; D006391:Hemangioma / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D007429:Intraocular Pressure; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis; D019572:Retinal Neoplasms / Q000175:diagnosis / Q000209:etiology*; D012189:Retrospective Studies; D058471:Subretinal Fluid; D014792:Visual Acuity; D018630:Vitreoretinopathy, Proliferative / Q000209:etiology; D014823:Vitreous Hemorrhage / Q000209:etiology; D055815:Young Adult
chemical_list: D019793:Fluorescein