title: Treatment outcome of osteosarcoma after bilateral retinoblastoma: a retrospective study of eight cases.
abstract: OBJECTIVE
  To analyse clinical characteristics and treatment outcomes of osteosarcoma that developed in survivors of bilateral retinoblastoma.
  
  
  METHODS
  Three institutions participated in this retrospective study. Among survivors of bilateral retinoblastoma who were diagnosed and treated between 1995 and 2012, 8 cases (4 male, 4 female) of osteosarcoma were identified. Medical records were thoroughly reviewed.
  
  
  RESULTS
  Median age at diagnosis of bilateral retinoblastoma was 8.5 months (range 1.4-18.4 months). Treatment modalities for retinoblastoma were: enucleation+chemotherapy+radiotherapy (n=6); chemotherapy combined with focal therapy (n=1); and chemotherapy+radiotherapy (n=1). Median radiotherapy dose was 46.5 Gy (range 45-54 Gy). Median age at diagnosis of osteosarcoma was 8.9 years (range 5.4-20.3 years). Median interval between retinoblastoma and osteosarcoma was 8.2 years (range 5.0-20.0 years). Tumour locations were femur (n=5), tibia (n=1), mandible (n=1), and nasal cavity (n=1). Two patients presented with lung metastasis. Seven patients received multimodal treatment, and treatment was refused in 1 patient. After diagnosis of osteosarcoma, the patients were followed for a median of 17.3 months (range 4.4-56.4 months). The 2-year overall survival and event-free survival rates were 56.3 ± 19.9% and 33.3 ± 18.0%, respectively. At the time of analysis, 5 patients remained alive, and 2 of them were on therapy. Of the 3 surviving patients without evidence of disease, 2 received high dose chemotherapy with autologous peripheral blood stem cell support.
  
  
  CONCLUSIONS
  Our data could be used as a basis for future studies aimed at reaching consensus about long term follow-up and treatment guidelines for this genetically susceptible group of patients.
authors.affiliation: Department of Pediatrics, Korea Cancer Center Hospital, Seoul, Republic of Korea.
authors.forename: Jun Ah
authors.initials: JA
authors.lastname: Lee
authors.affiliation: Department of Ophthalmology, Korea Cancer Center Hospital, Seoul, Republic of Korea.
authors.forename: Sang Yul
authors.initials: SY
authors.lastname: Choi
authors.affiliation: Division of Hematology/Oncology, Department of Pediatrics, Cancer Research Institute, Seoul National University, College of Medicine, Seoul, Republic of Korea.
authors.forename: Hyoung Jin
authors.initials: HJ
authors.lastname: Kang
authors.affiliation: Division of Hematology/Oncology, Department of Pediatrics, Cancer Research Institute, Seoul National University, College of Medicine, Seoul, Republic of Korea.
authors.forename: Ji Won
authors.initials: JW
authors.lastname: Lee
authors.affiliation: Division of Hematology/Oncology, Department of Pediatrics, Cancer Research Institute, Seoul National University, College of Medicine, Seoul, Republic of Korea Department of Pediatrics, Seoul National University Boramae Hospital, Seoul National University, College of Medicine, Seoul, Republic of Korea.
authors.forename: Hyery
authors.initials: H
authors.lastname: Kim
authors.affiliation: Department of Ophthalmology, Seoul National University, College of Medicine, Seoul, Republic of Korea.
authors.forename: Jeong Hun
authors.initials: JH
authors.lastname: Kim
authors.affiliation: Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University, School of Medicine, Seoul, Republic of Korea.
authors.forename: Ki Woong
authors.initials: KW
authors.lastname: Sung
authors.affiliation: Division of Hematology/Oncology, Department of Pediatrics, Cancer Research Institute, Seoul National University, College of Medicine, Seoul, Republic of Korea.
authors.forename: Hee Young
authors.initials: HY
authors.lastname: Shin
authors.affiliation: Division of Hematology/Oncology, Department of Pediatrics, Cancer Research Institute, Seoul National University, College of Medicine, Seoul, Republic of Korea.
authors.forename: Hyo Seop
authors.initials: HS
authors.lastname: Ahn
authors.affiliation: Division of Hematology/Oncology, Department of Pediatrics, Cancer Research Institute, Seoul National University, College of Medicine, Seoul, Republic of Korea.
authors.forename: Kyung Duk
authors.initials: KD
authors.lastname: Park
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects; D001859:Bone Neoplasms / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D059248:Chemoradiotherapy / Q000009:adverse effects; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D015353:Eye Enucleation; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D016609:Neoplasms, Second Primary / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D012516:Osteosarcoma / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D019572:Retinal Neoplasms / Q000473:pathology / Q000628:therapy*; D012175:Retinoblastoma / Q000473:pathology / Q000628:therapy*; D012189:Retrospective Studies; D013514:Surgical Procedures, Operative / Q000009:adverse effects; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
keywords: Child health (paediatrics); Neoplasia; Treatment other