原发性腹膜后脂肪肉瘤的发病机制及综合治疗  

Pathogenesis and comprehensive treatment of primary retroperitoneal liposarcoma

作　　者：李创坤 周晓 凌家瑜[1] 曾展涛 黄榕康[1] 王辉[1] LI Chuangkun;ZHOU Xiao;LING Jiayu;ZENG Zhantao;HUANG Rongkang;WANG Hui(The Sixth Affiliated Hospital,Sun Yat-sen University&Guangdong Provincial Institute of Gastroenterology&Guangdong Provincial Key Laboratory of Colorectal and Pelvic Floor Disease,Guangzhou 510655,P.R.China;Yanbu Hospital,Nanhai District of Foshan City,Foshan,Guangdong 528247,P.R.China)

机构地区：[1]中山大学附属第六医院,广东省胃肠病学研究所,广东省结直肠盆底疾病研究重点实验室,广州510655 [2]佛山市南海区盐步医院,广东佛山528247

出　　处：《中国普外基础与临床杂志》2020年第4期504-509,共6页Chinese Journal of Bases and Clinics In General Surgery

基　　金：广州市科技计划项目(项目编号:201704020059)。

摘　　要：目的了解原发性腹膜后脂肪肉瘤(PRLPS)的发病机制及综合治疗的研究进展,以期为临床医生诊疗提供依据。方法复习近年来关于PRLPS的病理分型、发病机制以及包括手术、放疗、化疗及分子靶向治疗在内的综合治疗研究进展的相关文献并加以综述。结果 PRLPS病理分型分为高分化、去分化、黏液样或圆细胞型、多形型及混合型5种。分子学发病机制主要是基因表达的变化,如MDM2与相关基因的协同作用、c-myc基因表达异常、Prune-nm23-H1机制、miRNA异常表达及FUS-CHOP融合基因的异常蛋白质产物调节肿瘤的生长。PRLRS的治疗包括肿瘤根治切除术、扩大切除术、姑息性切除术,辅之以放化疗以及分子靶向治疗。结论 PRLPS是一种复发率极高、较罕见的恶性肿瘤,但早期诊疗比较困难。随着对PRLPS分子机制的进一步研究,其治疗方式已转变为以手术切除治疗为主、辅以放化疗及分子靶向治疗的综合治疗方式。Objective To understand the pathogenesis and the research progress of comprehensive treatment of primary retroperitoneal liposarcoma(PRLPS)and to provide evidence for clinical diagnosis and treatment.Method The recent literatures on the pathological classification,pathogenesis of PRLPS,and comprehensive treatment including the surgery,radiotherapy,chemotherapy,and molecular targeted therapy were reviewed.Results The pathological types of PRLPS were highly differentiated,dedifferentiated,mucoid/round cell,polymorphic,and mixed.The main molecular pathogenesis was the synergistic effect of MDM2 with related genes,abnormal expressions of c-myc gene and microRNAs,Prune-nm23-H1 mechanism,and abnormal protein products of FUS-CHOP fusion gene which regulated the growth of tumor.The treatment of PRLRS included the radical resection,extended resection,and palliative resection combined with radiotherapy,chemotherapy,and molecular targeted therapy.Conclusions PRLPS is a rare malignant tumor with high recurrence rate,but early diagnosis and treatment are difficult.With the further study of the molecular mechanism of PRLPS,the treatment of PRLPS has been transformed into a comprehensive treatment based on surgery,adjuvant radiotherapy and chemotherapy,and molecular targeted therapy.

关 键 词：腹膜后脂肪肉瘤 发病机制 治疗 

分 类 号：R735.4[医药卫生—肿瘤]