纤维结构不良肉瘤变临床病理学分析

Sarcoma arising in fibrous dysplasia: a clinicopathological analysis

目的探讨纤维结构不良肉瘤变的临床病理特点及危险因素。方法收集上海交通大学附属第六人民医院2008年1月至2018年7月诊治的纤维结构不良肉瘤变病例18例, 回顾性分析发病的特点及组织学类型。应用IBM SPSS Statistics 19统计软件进行统计学分析。结果纤维结构不良肉瘤变, 男性11例, 女性7例, 男女比为1.57∶1.00, 发病年龄24~87岁, 平均年龄49岁, 股骨为好发部位。其中9例恶性成分为骨肉瘤, 3例为高级别肉瘤, 6例为低级别肉瘤。Logistic回归分析显示, 与多骨型纤维结构不良、复发性纤维结构不良病例相比, 年龄是肉瘤变独立的危险因素, Wals值为13.61(P<0.05), 比值比为12.82, 95%置信区间为3.31~49.70。结论纤维结构不良肉瘤变的临床表现不特异, 发病年龄超过40岁, 肉瘤变的风险增加约12倍。

Objective To study the clinicopathologic characteristics and risk factors of sarcoma arising in fibrous dysplasia.Methods A total of 18 cases were collected from January 2008 to July 2018 in Shanghai Jiaotong University Affiliated Sixth People′s Hospital.The characteristics and the histologic type were retrospectively reviewed.IBM SPSS 19 was used for statistical analysis.Results The male to female ratio of patients with fibrodysplastic sarcomatosis was 1.57∶1.00.The age of onset ranged from 24 to 87 years(mean 49 years).The long bones,especially the femur,were most frequently involved.Nine cases were osteosarcomas,three cases were high grade sarcoma and six cases were low grade sarcoma.Logistic regression analysis showed that age was an independent risk factor for sarcomatous change,compared with polyostotic or recurrent cases.Value of Wals was 13.61(P<0.05),and odds ratio was 12.82,95%confidence interval was 3.31-49.70.Conclusions Fibrodysplasia sarcomatosis is clinically nonspecific and the risk of sarcomatous changes increases approximately 12-fold when age of onset of fibrous dysplasia is over 40 years.