Objective: To explore the clinical characteristics of patients with pre-malignant or malignant transformation in ectopic endometrium of adenomyosis. Methods: A retrospective study was performed on 4 818 patients received surgical treatment in Peking Union Medical College Hospital and pathologically confirmed as adenomyosis from January 2010 to October 2016. The clinical features and prognosis of 30 patients among these with pre-malignant or malignant transformation in ectopic endometrium were reviewed. Results: A total of 4 818 patients pathologically diagnosed with adenomyosis. Among these,30 (0.62%,30/4 818) patients were diagnosed with pre-malignant or malignant transformation in foci of adenomyosis, including 16 (0.33%, 16/4 818) of atypical endometrial hyperplasia and 14 (0.29%, 14/4 818) of endometrioid carcinoma. The average age of 30 patients was (50.8±3.0) years (range: 27-65 years). Patients with atypical hyperplasia in foci of adenomyosis coexist with 13 cases of endometrioid carcinoma in situ, 1 case of borderline serous papilloma in eutopic endometrium, 1 case of atypical hyperplasia in eutopic endometrium, and 1 case of normal eutopic endometrium. Fourteen patients with endometrioid carcinoma in foci of adenomyosis coexist with endometrioid carcinoma in situ. Among the 27 patients with pre-malignant or malignant transformation in adenomyosis and coexisting with endometrioid carcinoma in situ, 23 of them were in International Federation of Gynecology and Obstetrics (FIGO) stage Ⅰa (85%, 23/27), 2 in stage Ⅰb (7%, 2/27), and 2 in stage Ⅲa (7%, 2/27). Seven percent (2/27) of patients had deep myometrial infiltration, and 7% (2/27) of patients had lymphovascular space invasion. The median follow-up time was 60 months (range: 32-104 months), and there were no recurrence or death. Conclusions: The incidence of atypical glandular hyperplasia or malignant transformation in foci of adenomyosis is rare, and it may be closely related to endometrial disease in situ. Pre-malignant or malignant change in foci of adenomyosis seems not to increase the risk of deep myometrial invasion, lymphovascular space invasion, and recurrence or mortality in patients of adenomyosis with endometrial adenocarcinoma.目的: 探讨异位内膜发生非典型增生或癌变的子宫腺肌病患者的临床病理特点、治疗及预后。 方法: 收集2010年1月—2016年10月间中国医学科学院北京协和医学院北京协和医院收治的手术后病理诊断中包含“子宫腺肌病”“子宫肌腺病”“子宫腺肌症”“子宫肌腺症”的患者共4 818例,其中异位内膜发生非典型增生或癌变的患者共30例,回顾性分析其临床病理特点、治疗及预后。 结果: (1)临床病理特点:4 818例子宫腺肌病患者中,其异位内膜发生非典型增生或癌变者共30例(0.62%,30/4 818),其中非典型增生、子宫内膜样癌分别为16、14例,各占0.33%(16/4 818)和0.29%(14/4 818)。30例异位内膜发生非典型增生或癌变患者的年龄为(50.8±3.0)岁(27~65岁);其中,16例异位内膜为非典型增生的患者合并在位内膜子宫内膜样癌13例,合并交界性浆液性乳头状瘤、局灶非典型增生、正常子宫内膜各1例,14例异位内膜为子宫内膜样癌的患者均合并在位内膜子宫内膜样癌。27例异位内膜非典型增生或癌变合并在位内膜子宫内膜样癌的子宫腺肌病患者中,其子宫内膜样癌的国际妇产科联盟(FIGO)手术病理分期Ⅰa期23例(85%,23/27),Ⅰb期2例(7%,2/27),Ⅲa期2例(7%,2/27);7%(2/27)的患者有深肌层浸润,7%(2/27)的患者有淋巴脉管间隙浸润。(2)治疗:以手术治疗为主,术后酌情辅以放化疗。30例患者中,13例行子宫全切除+双侧输卵管或附件切除术,包括术前诊断为子宫腺肌病1例、子宫内膜非典型增生7例、子宫内膜非典型增生不除外癌变5例;17例行子宫全切除+双侧附件切除+盆腔淋巴清扫术,包括术前诊断为子宫内膜非典型增生不除外癌变、术中剖视可疑子宫肌层浸润2例,子宫内膜样癌14例,以及1例附件区肿物待查患者,术中冰冻病理检查提示右侧卵巢子宫内膜样癌(术中同时行大网膜切除+阑尾切除术)。7例患者术后行辅助化疗和(或)放疗,包括Ⅰa期3例(其中腹水细胞学阳性、淋巴脉管间隙浸润阳性、G2各1例)、Ⅰb期2例、Ⅲa期2例。(3)预后:30例患者的中位随诊时间为60个月(32~104个月),随访期内无复发和死亡。 结论: 子宫腺肌病患者异位内膜非典型增生或癌变的发生率低,多合并子宫在位内膜病变。异位内膜发生非典型增生或癌变可能不增加子宫腺肌病合并子宫内膜癌患者的深肌层浸润、淋巴脉管间隙浸润、复发和死亡的风险。.