Abstract: Objective　To explore the clinical features and prognosis of prophylactic hysterectomy （PH） verus nonprophylactic hysterectomy（NPH） in patients with Lynch syndrome（LS）and Lynch syndrome-associated endometrial cancer（LS-EC），with a view to providing a reference for the treatment and prognosis improvement of this disease.Methods　16 patients with LS and 22 patients with LS-EC were involved from the First Hospital of Hebei Medical University from December 2014 to October 2016.Among them，16 voluntarily underwent PH （PH group），and other 22 underwent NPH（NPH group）．The baseline characteristics，results of quality of life assessment，histopathology and gene mutation analysis as well as survival rate of the two groups were collected.Results　Two groups showed significant differences in the time between diagnosis and surgery，and prevalence of symptoms associated with endometrial cancer（EC） （P<0.05）．With regard to histopathological findings，in the PH group，10 were found with abnormal results，including 5 with EC，3 with diffuse endometrial hyperplasia，1 with clear-cell endometrial cancer，and 1 with focal endometrial hyperplasia；the other 6 were found without endometrial cancer，including 3 with mild endometrial hyperplasia，2 with uterine fibroids，and 1 with uterine membrane atrophy；in the NPH group，19 were identified with endometrioid adenocarcinoma，2 with mucinous endometrial adenocarcinoma，1 with serous endometrial cancer；tumor location，status of lymph node metastasis，pathological type，pathological grading of endometrioid adenocarcinoma，and status of uterine muscle layer infiltration were similar in EC patients of both groups（P>0.05），but tumor diameter was less in EC patients of PH group（P<0.05）．Gene mutation analysis revealed that the mutation rates of hMSH2，hMSH6 and hPMS2 were similar in both groups（P>0.05），but mutation rate of hMLH1 was much higher in the PH group（P<0.05）．Up to 2017-03-01，27 survived and 11 died in all patients；PH group had higher survival rate than NPH group （χ2=4.900，P=0.027）．Conclusion　LS patients are usually accompanied by a history of cancer，and from diagnosis to longer operation time，but it can be reduced by PH.

Key words: Colorectal neoplasms, hereditary nonpolyposis；Endometrial neoplasms；Hysterectomy；Post-exposure prophylaxis

摘要： 目的　探究Lynch综合征（LS）患者预防性子宫切除（PH）与LS相关性子宫内膜癌（LS-EC）患者非预防性子宫切除（NPH）的临床特征及预后，为改善LS的预后提供指导。方法　选取2014年12月—2016年10月河北医科大学第一医院自愿行PH的LS患者16例为PH组，行NPH的LS-EC患者22例为NPH组。收集患者一般资料、病理组织学评估、基因突变分析及随访结果。结果　两组患者确诊LS至手术时间、子宫内膜癌（EC）症状比较，差异有统计学意义（P<0.05）。PH组病理结果显示：10例患者标本中有异常发现，其中5例为EC，3例为弥漫性增生，1例透明细胞癌，1例为局灶性增生；剩余6例患者中，3例内膜增生不活跃，2例有子宫肌瘤，1例子宫内膜萎缩。NPH组病理结果显示：内膜样腺癌19例，黏液性腺癌2例，浆液性腺癌1例。PH组EC患者肿瘤直径小于NPH组（P<0.05）；两组患者肿瘤位置、淋巴结转移情况、病理类型、内膜样腺癌病理分级、肌层浸润情况比较，差异无统计学意义（P>0.05）。PH组hMLH1基因突变率高于NPH组（P<0.05）；两组hMSH2、hMSH6、hPMS2基因突变率比较，差异无统计学意义（P>0.05）。截至2017-03-01，38例LS患者中，存活27例，死亡11例，PH组患者的生存率高于NPH组（χ2=4.900，P=0.027）。结论　LS患者多伴有肿瘤病史，并且从确诊至手术时间较长，而PH可改善LS患者的预后。

关键词: 结直肠肿瘤, 遗传性非息肉性, 子宫内膜肿瘤, 子宫切除术, 暴露后预防