title: High-dose chemotherapy followed by stem cell transplantation in the management of retinoblastoma: a systematic review.
abstract: OBJECTIVE
  In recent years, there has been an increasing role for stem cell transplantation in the management of retinoblastoma. The aim of this study was to systematically review the role high-dose chemotherapy followed by stem cell transplantation in the treatment of patients with metastatic or relapsed, trilateral or bilateral advanced retinoblastoma, and in patients with tumor at the surgical margin of the optic nerve and/or extrascleral extension.
  
  
  METHODS
  Systematic literature review.
  
  
  METHODS
  We performed an extensive PubMed database search on 25 February 2012 for studies describing the use of high-dose chemotherapy followed by stem cell transplantation in the management of patients with retinoblastoma.
  
  
  RESULTS
  We located 15 studies that met the inclusion criteria and that included 101 patients. Following treatment for metastatic and relapsed disease, 44 of 77 patients (57.1%) were alive with no evidence of disease at the time of follow-up. However, a higher rate of local relapse developed in patients with CNS metastases (73.1%), which dropped to 47.1% in patients who received thiotepa. In patients with trilateral or bilateral advanced retinoblastoma, 5 of 7 (71.4%) with reported outcome data were alive with no evidence of disease at the time of follow-up. In patients with tumor at the surgical margin of the optic nerve and/or extrascleral extension, 6 of 7 patients (85.7%) were alive with no evidence of disease at the time of follow-up.
  
  
  CONCLUSIONS
  Durable tumor control is possible in patients with non-CNS metastases, trilateral or bilateral advanced retinoblastoma, and in patients with tumor at the surgical margin of the optic nerve and/or extrascleral extension. Patients with CNS metastases require thiotepa to improve tumor control.
authors: Department of Radiation Oncology, King Hussein Cancer Center, Amman, Jordan.
authors: Imad
authors: I
authors: Jaradat
authors: Rasmi
authors: R
authors: Mubiden
authors: Ahmed
authors: A
authors: Salem
authors: Fawzi
authors: F
authors: Abdel-Rahman
authors: Iyad
authors: I
authors: Al-Ahmad
authors: Abdelatief
authors: A
authors: Almousa
mesh_terms: D000293:Adolescent; D000970:Antineoplastic Agents / Q000008:administration & dosage*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D006801:Humans; D019572:Retinal Neoplasms / Q000188:drug therapy / Q000473:pathology / Q000601:surgery / Q000628:therapy*; D012175:Retinoblastoma / Q000188:drug therapy / Q000473:pathology / Q000601:surgery / Q000628:therapy*; D033581:Stem Cell Transplantation; D013852:Thiotepa / Q000008:administration & dosage
chemical_list: D000970:Antineoplastic Agents; D013852:Thiotepa