title: Secondary Craniofacial Sarcomas Following Retinoblastoma: A Systematic Review.
abstract: OBJECTIVE
  We conducted the largest systematic review of individual patient data to characterize secondary craniofacial sarcomas following retinoblastoma.
  
  
  METHODS
  We conducted a systemic search of the PubMed databases and compiled a comprehensive literature review. Student t tests were used to evaluate differences between variables. Kaplan-Meier analysis was used to estimate survival. Statistical significance was assessed using a log-rank test.
  
  
  RESULTS
  We analyzed 220 cases of secondary craniofacial sarcomas, including 112 osteosarcomas. The average age (±SD) of onset for retinoblastoma was 1.20 ± 2.77 years. External-beam radiotherapy was delivered in 207 patients (94.0%) and chemotherapy was delivered in 53 patients (24.0%) patients. The latency period between retinoblastoma diagnosis and the onset of secondary sarcomas was 12 years. Cranial extension was found in 66 patients (30.0%). The median overall survival was worse with cranial extension (P = 0.0073). In cranial extended patients, the median survival in patients who received chemotherapy was 41 months, whereas patients who did not receive chemotherapy had a median survival of 12 months (P = 0.0020).
  
  
  CONCLUSIONS
  The risk of incidence of secondary sarcomas in retinoblastoma patients warrants longer follow-up periods. Moreover, chemotherapy should be considered as a potential treatment option for secondary cranial sarcomas following retinoblastoma.
authors.affiliation: Laboratory of Molecular Target Therapy for Cancer, Graduate School for Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan. Electronic address: ryaman@koto.kpu-m.ac.jp.
authors.forename: Ryuya
authors.initials: R
authors.lastname: Yamanaka
authors.affiliation: Laboratory of Molecular Target Therapy for Cancer, Graduate School for Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan.
authors.forename: Azusa
authors.initials: A
authors.lastname: Hayano
mesh_terms: D001859:Bone Neoplasms / Q000175:diagnosis / Q000401:mortality; D005147:Facial Bones / Q000473:pathology; D006801:Humans; D016609:Neoplasms, Second Primary / Q000175:diagnosis* / Q000401:mortality; D012516:Osteosarcoma / Q000175:diagnosis / Q000401:mortality; D012175:Retinoblastoma / Q000175:diagnosis* / Q000401:mortality; D012509:Sarcoma / Q000175:diagnosis* / Q000401:mortality; D012888:Skull Neoplasms / Q000175:diagnosis* / Q000401:mortality; D015996:Survival Rate / Q000639:trends
keywords: Chemotherapy; Cranial extension; Craniofacial sarcomas; Radiation therapy; Radiation-associated sarcoma; Retinoblastoma