title: Treatment of overt extraocular retinoblastoma.
abstract: BACKGROUND
  Overt extraocular retinoblastoma is common in developing countries and little information about its treatment is available. The aim of this study is to report our experience in the treatment of these cases using a uniform approach.
  
  
  METHODS
  Patients with overt extraocular retinoblastoma including orbital extension, preauricular lymph node invasion and/or metastatic disease on diagnosis or after extraocular relapse admitted to the Hospital JP Garrahan from August 1987 to December 2000 were retrospectively reviewed. Patients were treated according to two different protocols (1987-1993 and 1994-2000). Treatment included: neoadjuvant combination chemotherapy followed by limited surgery in case of orbital extension (enucleation or resection of residual orbital mass) and adjuvant chemotherapy and radiotherapy. Chemotherapy included cyclophosphamide, vincristine, etoposide, doxorubicin (in protocol 87), idarubicin (in protocol 94), cisplatin (in protocol 87), and carboplatin (in protocol 94).
  
  
  RESULTS
  Forty-one patients were included. Fifteen of them had orbital or preauricular disease and had a 5-year event-free survival (pEFS) of 84%. Twenty-six had distant metastatic disease and non survived 5-years. One patient died of toxicity and one died in complete remission. One patient had a secondary leukemia. The remaining adverse events included CNS and/or systemic relapse.
  
  
  CONCLUSIONS
  This treatment strategy was highly efficacious for patients with orbital and/or lymph node extension. Orbital exenteration is not necessary for these patients. Those patients with distant metastatic or CNS disease were not curable with this approach.
authors.affiliation: Department of Hematology/Oncology, Hospital de Pediatría JP Garrahan, Combate de los Pozos 1881, Buenos Aires, 1245, Argentina. gchantada@garrahan.gov.ar
authors.forename: Guillermo
authors.initials: G
authors.lastname: Chantada
authors.forename: Adriana
authors.initials: A
authors.lastname: Fandiño
authors.forename: Sandra
authors.initials: S
authors.lastname: Casak
authors.forename: Julio
authors.initials: J
authors.lastname: Manzitti
authors.forename: Elsa
authors.initials: E
authors.lastname: Raslawski
authors.forename: Enrique
authors.initials: E
authors.lastname: Schvartzman
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D017024:Chemotherapy, Adjuvant / Q000379:methods; D003131:Combined Modality Therapy / Q000379:methods; D003906:Developing Countries; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D020360:Neoadjuvant Therapy / Q000379:methods; D009361:Neoplasm Invasiveness; D009362:Neoplasm Metastasis; D013508:Ophthalmologic Surgical Procedures / Q000379:methods; D018714:Radiotherapy, Adjuvant / Q000379:methods; D019572:Retinal Neoplasms / Q000188:drug therapy* / Q000473:pathology / Q000532:radiotherapy* / Q000601:surgery; D012175:Retinoblastoma / Q000188:drug therapy* / Q000473:pathology / Q000532:radiotherapy* / Q000601:surgery; D012189:Retrospective Studies; D016019:Survival Analysis; D013997:Time Factors; D016896:Treatment Outcome