107例韧带样纤维瘤病临床病理学特征及预后分析  

Clinicopathologic features and prognostic factors of desmoid-type fibromatosis:An analysis of 107 cases

作　　者：王晓聪 宋欣[1] 刘晋红[1] 孙海波[1] 李金龙[1] 石怀银[1] 

机构地区：[1]解放军总医院病理科,北京100853

出　　处：《解放军医学院学报》2017年第11期1033-1036,1039,共5页Academic Journal of Chinese PLA Medical School

摘　　要：目的探讨韧带样纤维瘤病(侵袭性纤维瘤病)的临床病理学特征及影响其预后的相关因素。方法收集本院2005-2015年收治的107例韧带样纤维瘤病患者的临床资料,分析该病的临床病理学特征,进行P53和β-catenin免疫组织化学染色,回顾性分析影响肿瘤复发的因素。结果 107例患者中男性49例(45.8%),女性58例(54.2%),平均年龄27.6(1~78)岁。肿瘤位于上肢8例,下肢26例,躯干62例,头颈部11例。肿瘤平均直径为8.9(1.2~30.0)cm。中位随访时间为6.4(3.1~11.0)年。107例患者总复发率为47.7%(51/107),复发时间为术后2~70个月,中位复发时间为12个月。单因素分析发现切缘阳性(P=0.000)、病变位于上肢(P=0.001)、β-catenin核阳性表达(P=0.026)以及P53蛋白阳性表达(P=0.001)可提示患者预后不良。多因素分析显示切缘阳性(P=0.000)、病变位于上肢(P=0.003)、P53蛋白阳性表达(P=0.000)是患者术后复发的独立影响因素。结论手术切缘阳性是肿瘤复发的危险因素,P53蛋白阳性表达提示患者肿瘤复发的可能性大,需术后进一步治疗。Objective To study the clinicopathological features and risk factors for the recurrence of desmoid-type fibromatosis（aggressive fibromatosis）. Methods Clinical data about 107 desmoid-type fibromatosis patients who admitted to Chinese PLA General Hospital from 2005 to 2015 were collected. The clinicopathological features and the expressions of P53 and β-catenin were used to analyze the factors influencing the prognosis of desmoid-type fibromatosis retrospectively. Results Of the 107 cases, 49 males（45.8%） and 58 females（54.2%） were included with mean age of 27.6 years（ranging from 1 to 78 years）. The tumors located at upper extremity in 8 cases, lower extremity in 26 cases, trunk in 62 cases, head and neck in 11 cases. The mean diameter of the tumors was 8.9 cm（ranging from 1.2 to 30.0 cm）. The median follow-up time was 6.4 years（ranging from 3.1 to 11.0 years）. Of the 107 cases, 51（47.7%） patients recurred after surgical resection. The recurrence develped in 2 to 70 months with median of 12 months. Univariate analysis revealed that surgical margin（P=0.000）, tumor location（P=0.001）, nuclear β-catenin（P=0.026） and P53（P=0.001） expression were the factors influencing tumor recurrence. Multivariate analysis revealed that surgical margin（P=0.000）, tumor location（P=0.003） and P53 expression（P=0.000） were independent prognostic factors. Conclusion Positive surgical margin is a risk factor for the recurrence of desmoid-type fibromatosis. The expression of P53 is a poor prognostic factor, suggesting further treatment after surgery.

关 键 词：韧带样纤维瘤病 复发 免疫组化 β连接蛋白 P53蛋白 

分 类 号：R738[医药卫生—肿瘤]