腹腔内侵袭性纤维瘤病临床治疗回顾

Clinical treatment review of intraperitoneal aggressive fibromatosis

目的探讨腹内型侵袭性纤维瘤病的临床特点、治疗方法和预后。方法回顾性分析2007年1月～2017年6月武汉大学人民医院收治的26例腹内型侵袭性纤维瘤病患者的临床资料,所有患者均经病理诊断证实。分析接受根治性手术患者和仅行放化疗患者的生存率,并将肿瘤直径≥10 cm组(8例)与肿瘤直径<10 cm(18例)组分别采用Kaplan-Meier法绘制生存曲线,两组生存率比较采用χ~2检验,生存曲线采用Log-rank检验。结果 26例患者术前均行CT或MRI检查,仅1例考虑为腹腔内侵袭性纤维瘤病。22例接受根治性切除术的患者中,随访期间仅1例死亡,总生存率为95.45%;而未能切除仅行放化疗的4例患者中,随访期间有3例死亡,总生存率为25.00%,两组总生存率比较,差异有高度统计学意义(P <0.01)。肿瘤直径≥10 cm组与肿瘤直径<10 cm组的预后比较,差异有统计学意义(P=0.033)。结论腹内型侵袭性纤维瘤病术前诊断困难,初治时是否首选手术治疗、肿瘤大小、能否行根治性切除是影响其预后的关键性因素。

Objective To investigate the clinical characteristics,treatment and prognosis of intraperitoneal aggressive fibromatosis.Methods The clinical data of26patients with intraperitoneal aggressive fibromatosis in Renmin Hospital of Wuhan University from January2007to June2017were analyzed retrospectively.All patients were confirmed by pathological diagnosis.The survival rate was analyzed between the patients undergoing radical surgery and those only undergoing radiotherapy or chemotherapy,and the survival curve was drawn by Kaplan-Meier method for the group with the tumor diameter larger than10cm(8cases)and the group with the tumor diameter less than10cm(18cases)respectively.The survival rate of two groups were compared withχ2test and the survival curve was tested by Log-rank test.Results All the 26 patients were examined by CT or MRI before operation,only1case was considered as intraperitoneal aggressive fibromatosis.Among22patients who underwent radical resection,only1case dead in follow-up period,the overall survival rate was95.45%;among4patients who only accepted radiotherapy or chemotherapy,3cases dead in follow-up period,overall survival rate was25.00%,the difference in overall survival rate between the two groups was highly statistically significant(P<0.01).There was significant prognosis difference between the groups of tumor diameter larger than10cm and less than10cm(P=0.033).Conclusion Intraperitoneal aggressive fibromatosis is difficult to diagnose before operation.Whether surgical treatment is the first choice,whether to perform radical resection and tumor size are the key factors affecting prognosis.