Open AccessIncidence, Predictive Factors, and Prognosis of Chondrosarcoma in Patients with Ollier Disease and Maffucci Syndrome: An International Multicenter Study of 161 Patients
Author(s) -
Verdegaal Suzan H.M.,
Bovée Judith V.M.G.,
Pansuriya Twinkal C.,
Grimer Robert J.,
Ozger Harzem,
Jutte Paul C.,
San Julian Mikel,
Biau David J.,
Geest Ingrid C.M.,
Leithner Andreas,
Streitbürger Arne,
Klenke Frank M.,
Gouin Francois G.,
Campanacci Domenico A.,
MarecBerard Perrine,
Hogendoorn Pancras C.W.,
Brand Ronald,
Taminiau Antonie H.M.
Publication year - 2011
Publication title -
the oncologist
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.176
H-Index - 164
eISSN - 1549-490X
pISSN - 1083-7159
DOI - 10.1634/theoncologist.2011-0200
Subject(s) - chondrosarcoma , medicine , incidence (geometry) , pelvis , axial skeleton , enchondroma , retrospective cohort study , disease , odds ratio , surgery , dermatology , radiology , anatomy , physics , optics
Learning Objectives After completing this course, the reader will be able to: Describe major enchondroma distribution patterns that were identified in this study. Identify variables that are predictive for the secondary transformation of enchondroma over the lifetime of individuals with Ollier disease or Maffucci syndrome.This article is available for continuing medical education credit at CME.TheOncologist.comBackground. 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. Method. 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.