title: Clinical characteristics and prognosis of osteosarcoma in young children: a retrospective series of 15 cases.
abstract: BACKGROUND
  Osteosarcoma is the most common primary bone malignancy in childhood and adolescence. However, it is very rare in children under 5 years of age. Although studies in young children are limited in number, they all underline the high rate of amputation in this population, with conflicting results being recently reported regarding their prognosis.
  
  
  METHODS
  To enhance knowledge on the clinical characteristics and prognosis of osteosarcoma in young children, we reviewed the medical records and histology of all children diagnosed with osteosarcoma before the age of five years and treated in SFCE (Société Française des Cancers et leucémies de l'Enfant) centers between 1980 and 2007.
  
  
  RESULTS
  Fifteen patients from 7 centers were studied. Long bones were involved in 14 cases. Metastases were present at diagnosis in 40% of cases. The histologic type was osteoblastic in 74% of cases. Two patients had a relevant history. One child developed a second malignancy 13 years after osteosarcoma diagnosis.Thirteen children received preoperative chemotherapy including high-dose methotrexate, but only 36% had a good histologic response. Chemotherapy was well tolerated, apart from a case of severe late convulsive encephalopathy in a one-year-old infant. Limb salvage surgery was performed in six cases, with frequent mechanical and infectious complications and variable functional outcomes.Complete remission was obtained in 12 children, six of whom relapsed. With a median follow-up of 5 years, six patients were alive in remission, seven died of their disease (45%), in a broad range of 2 months to 8 years after diagnosis, two were lost to follow-up.
  
  
  CONCLUSIONS
  Osteosarcoma seems to be more aggressive in children under five years of age, and surgical management remains a challange.
authors.affiliation: 1Department of Pediatric Oncology, Institut Curie, Paris, France.
authors.forename: Maud A M
authors.initials: MA
authors.lastname: Guillon
authors.forename: Pierre M J
authors.initials: PM
authors.lastname: Mary
authors.forename: Laurence
authors.initials: L
authors.lastname: Brugière
authors.forename: Perrine
authors.initials: P
authors.lastname: Marec-Bérard
authors.forename: Hélène D
authors.initials: HD
authors.lastname: Pacquement
authors.forename: Claudine
authors.initials: C
authors.lastname: Schmitt
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Guinebretière
authors.forename: Marie-Dominique P
authors.initials: MD
authors.lastname: Tabone
mesh_terms: D000671:Amputation, Surgical; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001859:Bone Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D002675:Child, Preschool; D003131:Combined Modality Therapy; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D012516:Osteosarcoma / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D011379:Prognosis; D012189:Retrospective Studies; D016896:Treatment Outcome