title: Diffuse Anterior Retinoblastoma with Globe Salvage and Visual Preservation in 3 Consecutive Cases.
abstract: OBJECTIVE
  Diffuse anterior retinoblastoma is an exquisitely rare variant of retinoblastoma in which the tumor resides in the anterior segment of the eye, without apparent retinal involvement. Previously published cases have been managed with enucleation. We describe globe salvage and visual preservation in 3 consecutive cases using chemotherapy and radiotherapy.
  
  
  METHODS
  Retrospective case series.
  
  
  METHODS
  Three children with diffuse anterior retinoblastoma.
  
  
  METHODS
  Plaque radiotherapy plus intravenous chemotherapy.
  
  
  METHODS
  Globe and vision preservation.
  
  
  RESULTS
  The mean patient age at presentation elsewhere was 5.7 years (median, 7; range, 3-7 years). There were 2 white female patients and 1 African American male patient. The initial observation by parents/caregiver was reduced vision (n = 1), red eye (n = 1), or cloudy eye (n = 1), and the initial finding by physician was iris tumor (n = 2) or hyphema (n = 1). Referring diagnosis was iris melanoma (n = 1), infectious endotheliitis (n = 1), and possible tumor (nonspecified) (n = 1). At our evaluation, visual acuity was 20/50 to 20/60 (n = 2) and fix no follow (n = 1). In all cases, the opposite eye was normal. Mean intraocular pressure was 20 mm Hg (median, 16; range, 15-30 mmHg). Our examination revealed solid iris tumor (n = 3), ciliary body involvement (n = 2), and anterior chamber seeding (n = 3). In no case was there choroidal or retinal tumor, vitreous seed or subretinal seed, or extrascleral extension. Clear corneal fine-needle aspiration biopsy confirmed the diagnosis as retinoblastoma in each case. Treatment included plaque radiotherapy (n = 3) plus additional systemic chemotherapy (n = 2). At mean follow-up of 35 months (median, 34; range, 20-51 months), there has been no recurrence, extrascleral extension, enucleation, metastasis, or death. In all 3 cases, cataract surgery was necessary at a mean interval of 16 months after complete and stable regression of retinoblastoma.
  
  
  CONCLUSIONS
  The rare diffuse anterior form of retinoblastoma can be managed with globe-salvaging alternatives and with visual preservation in selected cases.
authors.affiliation: Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania. Electronic address: carolshields@gmail.com.
authors.forename: Carol L
authors.initials: CL
authors.lastname: Shields
authors.affiliation: Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: Sara E
authors.initials: SE
authors.lastname: Lally
authors.affiliation: Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: Fairooz P
authors.initials: FP
authors.lastname: Manjandavida
authors.affiliation: The Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Ann M
authors.initials: AM
authors.lastname: Leahey
authors.affiliation: Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
authors.forename: Jerry A
authors.initials: JA
authors.lastname: Shields
mesh_terms: D000867:Anterior Chamber / Q000473:pathology*; D000970:Antineoplastic Agents / Q000627:therapeutic use; D044963:Biopsy, Fine-Needle; D001918:Brachytherapy; D002648:Child; D002675:Child, Preschool; D002924:Ciliary Body / Q000473:pathology*; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D015811:Iris Neoplasms / Q000473:pathology* / Q000503:physiopathology / Q000628:therapy; D008297:Male; D009366:Neoplasm Seeding; D012175:Retinoblastoma / Q000473:pathology* / Q000503:physiopathology / Q000628:therapy; D012189:Retrospective Studies; D014604:Uveal Neoplasms / Q000473:pathology* / Q000503:physiopathology / Q000628:therapy; D014792:Visual Acuity / Q000502:physiology
chemical_list: D000970:Antineoplastic Agents