title: NEWLY DIAGNOSED ACTIVE RETINOBLASTOMA IN ADULTS.
abstract: OBJECTIVE
  To describe the clinical features, treatment, and outcome of retinoblastoma in adults.
  
  
  METHODS
  Retrospective case series.
  
  
  RESULTS
  The mean age at initial presentation of retinoblastoma was 30 years (median, 26 years; range, 22-48 years). There were four males and four females, and all manifested unilateral retinoblastoma. The mean duration of symptoms was 22 months (median, 12 months; range, 1-100 months). Six patients had intraocular retinoblastoma, and 2 had secondary orbital involvement. The eyes with intraocular retinoblastoma were classified according to the International Classification of Retinoblastoma as Group D (n = 3) or Group E (n = 3). The primary treatment for intraocular retinoblastoma (n = 6) included systemic chemotherapy (n = 1), external beam radiotherapy (n = 2), and enucleation (n = 3). Secondary treatment for tumor recurrence included enucleation (n = 2), and combination of intraarterial chemotherapy, intravitreal chemotherapy, and plaque radiotherapy (n = 1). The eyes with orbital extension of retinoblastoma were classified according to the International Retinoblastoma Staging System as Stage 3a (n = 2). The primary treatment for those with orbital extension of retinoblastoma included multimodality treatment (combination of systemic chemotherapy, orbital exenteration, and external beam radiotherapy). Systemic metastasis and related death occurred in one case.
  
  
  CONCLUSIONS
  Retinoblastoma in adults is uncommon. Active tumor in this age group is usually advanced, necessitating enucleation and/or orbital exenteration.
authors.affiliation: *Institute for Eye Cancer, L. V. Prasad Eye Institute, Hyderabad, India; and †Ocular Oncology Service, Wills Eye Hospital, Philadelphia, Pennsylvania.
authors.forename: Swathi
authors.initials: S
authors.lastname: Kaliki
authors.forename: Carol L
authors.initials: CL
authors.lastname: Shields
authors.forename: Adit
authors.initials: A
authors.lastname: Gupta
authors.forename: Dilip K
authors.initials: DK
authors.lastname: Mishra
authors.forename: Chandana
authors.initials: C
authors.lastname: Das
authors.forename: Emil A T
authors.initials: EA
authors.lastname: Say
authors.forename: Jerry A
authors.initials: JA
authors.lastname: Shields
mesh_terms: D000328:Adult; D000970:Antineoplastic Agents / Q000008:administration & dosage*; D003131:Combined Modality Therapy; D015353:Eye Enucleation; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D019572:Retinal Neoplasms / Q000175:diagnosis / Q000532:radiotherapy / Q000628:therapy*; D012175:Retinoblastoma / Q000175:diagnosis / Q000532:radiotherapy / Q000628:therapy*; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D000970:Antineoplastic Agents