Incidence, predictive factors, and prognosis of chondrosarcoma in patients with Ollier disease and Maffucci syndrome: an international multicenter study of 161 patients

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; Van Der Geest, Ingrid C. M; Leithner, Andreas; Streitbürger, Arne; Klenke, Frank M; Gouin, Francois G; Campanacci, Domenico Andrea; Marec Berard, Perrine; Hogendoorn, Pancras C. W; Brand, Ronald; Taminiau, Antonie H. M.

doi:10.1634/theoncologist.2011-0200

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.

Incidence, predictive factors, and prognosis of chondrosarcoma in patients with Ollier disease and Maffucci syndrome: an international multicenter study of 161 patients / 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; van der Geest, Ingrid C M; Leithner, Andreas; Streitbürger, Arne; Klenke, Frank M; Gouin, Francois G; Campanacci, Domenico A; Marec-Berard, Perrine; Hogendoorn, Pancras C W; Brand, Ronald; Taminiau, Antonie H M. - In: THE ONCOLOGIST. - ISSN 1083-7159. - STAMPA. - 16:(2011), pp. 1771-1779. [10.1634/theoncologist.2011-0200]