title: Incidence, predictive factors, and prognosis of chondrosarcoma in patients with Ollier disease and Maffucci syndrome: an international multicenter study of 161 patients.
abstract: BACKGROUND
  Enchondromatosis is characterized by the presence of multiple benign cartilage lesions in bone. While Ollier disease is typified by multiple enchondromas, in Maffucci syndrome these are associated with hemangiomas. Studies evaluating the predictive value of clinical symptoms for development of secondary chondrosarcoma and prognosis are lacking. This multi-institute study evaluates the clinical characteristics of patients, to get better insight on behavior and prognosis of these diseases.
  
  
  METHODS
  A retrospective study was conducted using clinical data of 144 Ollier and 17 Maffucci patients from 13 European centers and one national databank supplied by members of the European Musculoskeletal Oncology Society.
  
  
  RESULTS
  Patients had multiple enchondromas in the hands and feet only (group I, 18%), in long bones including scapula and pelvis only (group II, 39%), and in both small and long/flat bones (group III, 43%), respectively. The overall incidence of chondrosarcoma thus far is 40%. In group I, only 4 patients (15%) developed chondrosarcoma, in contrast to 27 patients (43%) in group II and 26 patients (46%) in group III, respectively. The risk of developing chondrosarcoma is increased when enchondromas are located in the pelvis (odds ratio, 3.8; p = 0.00l).
  
  
  CONCLUSIONS
  Overall incidence of development of chondrosarcoma is 40%, but may, due to age-dependency, increase when considered as a lifelong risk. Patients with enchondromas located in long bones or axial skeleton, especially the pelvis, have a seriously increased risk of developing chondrosarcoma, and are identified as the population that needs regular screening on early detection of malignant transformation.
authors.affiliation: Leiden University Medical Center, Orthopedic Surgery, Leiden, The Netherlands. shmverdegaal@hotmail.com
authors.forename: Suzan H M
authors.initials: SH
authors.lastname: Verdegaal
authors.forename: Judith V M G
authors.initials: JV
authors.lastname: Bovée
authors.forename: Twinkal C
authors.initials: TC
authors.lastname: Pansuriya
authors.forename: Robert J
authors.initials: RJ
authors.lastname: Grimer
authors.forename: Harzem
authors.initials: H
authors.lastname: Ozger
authors.forename: Paul C
authors.initials: PC
authors.lastname: Jutte
authors.forename: Mikel
authors.initials: M
authors.lastname: San Julian
authors.forename: David J
authors.initials: DJ
authors.lastname: Biau
authors.forename: Ingrid C M
authors.initials: IC
authors.lastname: van der Geest
authors.forename: Andreas
authors.initials: A
authors.lastname: Leithner
authors.forename: Arne
authors.initials: A
authors.lastname: Streitbürger
authors.forename: Frank M
authors.initials: FM
authors.lastname: Klenke
authors.forename: Francois G
authors.initials: FG
authors.lastname: Gouin
authors.forename: Domenico A
authors.initials: DA
authors.lastname: Campanacci
authors.forename: Perrine
authors.initials: P
authors.lastname: Marec-Berard
authors.forename: Pancras C W
authors.initials: PC
authors.lastname: Hogendoorn
authors.forename: Ronald
authors.initials: R
authors.lastname: Brand
authors.forename: Antonie H M
authors.initials: AH
authors.lastname: Taminiau
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001859:Bone Neoplasms / Q000150:complications*; D002357:Cartilage Diseases / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D002813:Chondrosarcoma / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology; D004687:Enchondromatosis / Q000150:complications* / Q000473:pathology; D005060:Europe / Q000453:epidemiology; D005260:Female; D006391:Hemangioma / Q000150:complications*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D012878:Skin Neoplasms / Q000150:complications*