MRKH综合征合并卵巢恶性肿瘤一例  

A Case Report of MRKH Syndrome Complicated with Ovarian Malignant Tumor

作　　者：区晓榆 曾宇华 陈燕芬[3] 谢琳玲 曾蕾[4] 卢如玲[4] OU Xiao-yu;ZENG Yu-hua;CHEN Yan-fen;XIE Lin-ling;ZENG Lei;LU Ru-ling(The First Clinical Medical College of Guangzhou University of Chinese Medicine,Guangzhou 510405,China;Hunan Provincal Maternal and Child Health Care Hospital,Changsha 410011,China;Guangzhou University of Chinese Medicine,Guangzhou 510405,China;The First Affiliated Hospital of Guangzhou University of Chinese Medicine,Guangzhou 510405,China)

机构地区：[1]广州中医药大学第一临床医学院,510405 [2]湖南省妇幼保健院 [3]广州中医药大学 [4]广州中医药大学第一附属医院

出　　处：《国际生殖健康／计划生育杂志》2024年第2期121-126,共6页Journal of International Reproductive Health/Family Planning

摘　　要：MRKH综合征(Mayer-Rokitansky-Küster-Hauser syndrome)是一种较为罕见的先天性女性生殖器官发育障碍综合征,以无阴道及子宫发育不全为特征。该病患者具有发生盆腔合并症的风险,但由于其发病率低,治疗上缺乏经验总结,容易发生误诊漏诊。报告1例MRKH综合征合并巨大卵巢浆液性乳头状癌病例的诊治情况。该类患者通常拥有正常的卵巢结构,妇科医生应当警惕卵巢病变的风险,术前配合磁共振成像检查可提高诊断敏感性,通过腹腔镜手术可更清晰地明确盆腔情况同时降低手术损伤;年轻的卵巢癌患者可考虑保留健侧卵巢以保证内源性激素,而残基子宫则建议切除以降低病变风险。该例患者术后肝功能明显异常,最终予诺雷得抑制卵巢功能以择期启动化疗;卵巢癌化疗方案仍适用于该类患者且随访疗效满意。通过结合文献分析,旨在提高妇科医生对MRKH综合征患者盆腔合并症的认识和警觉。Mayer-Rokitansky-Küster-Hauser(MRKH)syndrome is a rare congenital disorder of female reproductive development,resulting in vaginal aplasia and uterine hypoplasia.Patients with MRKH syndrome are at the risk of pelvic complications.Due to the low incidence and limited treatment experience,MRKH syndrome is prone to be misdiagnosed.A case of MRKH syndrome complicated with giant ovarian plasma papillary carcinoma was presented.The patient with MRKH syndrome usually has normal structure of ovary,that is why gynecologists should be more alerted to the risk of ovarian pathology.Preoperative MRI can improve the diagnostic sensitivity,and laparoscopic surgery can clarify the pelvis and reduce surgical injuries.In young patients with ovarian cancer,preservation of the healthy ovary can be considered to ensure endogenous hormones,while resection of the stumpy uterus can be recommended to reduce the risk of lesions.In this case,the liver function after operation was obviously abnormal,and noladex was finally given to inhibit the ovarian function in order to initiate chemotherapy at an elective stage.The chemotherapy regimen for ovarian cancer should be still applicable and the follow-up results be satisfactory in this case.This case report with literature review will increase the awareness and alertness of gynecologists to pelvic comorbidities in patients with MRKH syndrome.

关 键 词：MRKH综合征 卵巢肿瘤 先天畸形 诊断 治疗 病例报告 

分 类 号：R737.31[医药卫生—肿瘤] R711.1[医药卫生—临床医学]