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Incidência, fatores preditivos e prognóstico de condrossarcoma em pacientes com doença de Ollier e a síndrome de Maffucci: um estudo multicêntrico internacional de 161 pacientes

Postado em: 31/07/2012

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

Autor(es): Verdegaal SH; Bovée JV; Pansuriya TC; Grimer RJ; Ozger H; Jutte PC; San Julian M; Biau DJ; van der Geest IC; Leithner A; Streitbürger A; Klenke FM; Gouin FG; Campanacci DA; Marec-Berard P; Hogendoorn PC; Brand R; Taminiau AH
Fonte: Oncologist; 16(12): 1771-9, 2011.
Artigo [MEDLINE PMID: 22147000 ] Idioma: Inglês
Tipo de publicação: Artigo de Revista; Estudo Multicêntrico; Research Support, Non-U.S. Gov't

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.

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.
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