Incidence, Predictive Factors, and Prognosis of Chondrosarcoma in Patients with Ollier Disease and Maffucci Syndrome: An International Multicenter Study of 161 Patients
Incidence, Predictive Factors, and Prognosis of Chondrosarcoma in Patients with Ollier Disease and Maffucci Syndrome: An International Multicenter Study of 161 Patients
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DOI：
10.1634/theoncologist.2011-0200
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发表时间：
2011-12-01
期刊：
影响因子：
5.8
通讯作者：
Taminiau, Antonie H. M.
中科院分区：
文献类型：
--
作者：
Verdegaal, Suzan H. M.;Bovee, Judith V. M. G.;Taminiau, Antonie H. M.
背景内生软骨瘤病的特点是骨内存在多个良性软骨病变。虽然奥利耶病的典型表现是多发性内生软骨瘤，但在马富奇综合征中，这些内生软骨瘤与血管瘤有关。缺乏临床症状对继发性软骨肉瘤发展和预后的预测价值的研究。这项多机构研究评估了患者的临床特征，以更好地了解这些疾病的行为和预后。本研究采用来自欧洲13个中心的144名Ollier和17名Maffucci患者的临床资料和欧洲肌肉骨骼肿瘤学会成员提供的一个国家数据库进行回顾性研究。患者的多发性内生软骨瘤分别位于手和脚（第一组，18%）、长骨（包括肩胛骨和骨盆）（第二组，39%）以及小骨和长/扁平骨（第三组，43%）。到目前为止，软骨肉瘤的总发病率为40%。在第一组中，只有4名患者（15%）发展成软骨肉瘤，而第二组和第三组分别有27名患者（43%）和26名患者（46%）。当内生软骨瘤位于骨盆时，发生软骨肉瘤的风险增加（优势比，3.8; p = 0.001）。软骨肉瘤的总体发病率为40%，但由于年龄依赖性，当被认为是终身风险时，可能会增加。位于长骨或中轴骨，特别是骨盆的内生软骨瘤患者发生软骨肉瘤的风险严重增加，并被确定为需要定期筛查以早期发现恶性转化的人群。肿瘤学家2011;16：1771-1779
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 = 0.001).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. The Oncologist 2011;16:1771-1779