Peutz-Jeghers综合征合并多发妇科肿瘤一例

Peutz-Jeghers Syndrome with Multiple Gynecological Tumors: A Case Report

Peutz-Jeghers综合征是一种常染色体显性遗传病,常伴发多种妇科肿瘤,且肿瘤类型较为罕见,在临床中易出现漏诊、误诊。现报告1例大连医科大学附属第一医院收治的Peutz-Jeghers综合征患者合并卵巢环状小管性索瘤、卵巢黏液性肿瘤和宫颈微偏腺癌的病例。患者以盆腔包块首次就诊,经卵巢肿瘤剥除术后诊断为卵巢黏液性囊腺瘤,并发微小的卵巢环状小管性索瘤,但经2次肿瘤剥除术后均短期内复发,最终进展为卵巢黏液性囊腺癌,第3次手术切除子宫后偶然发现同时存在宫颈微偏腺癌。回顾该患者的临床特征、诊治过程及术后随访情况,以提高临床医生对Peutz-Jeghers综合征相关妇科肿瘤的认识。

Peutz-Jeghers syndrome is an autosomal dominant genetic disease,often accompanied by a variety of gynecological tumors,and the tumor type is relatively rare,which is prone to misdiagnosis and missed diagnosis in clinical practice.We report a case of Peutz-Jeghers syndrome with ovarian sex cord tumor with annular tubules,ovarian mucinous tumor and cervical minimal deviation adenocarcinoma admitted to the First Affiliated Hospital of Dalian Medical University.The patient presented to the hospital for the first time with pelvic mass and was diagnosed as ovarian mucinous cystadenoma after ovarian tumor stripping.It was complicated with microscopic ovarian sex cord tumor with annular tubules,but the tumor recurred within a short time after the removal of the tumor twice,and eventually progressed to ovarian mucinous cystadenocarcinoma.The third operation was accidentally found to be accompanied by cervical minimal deviation adenocarcinoma.The clinical features,diagnosis and treatment process and postoperative follow-up of the patient are reviewed to improve clinicians′cognition of gynecologic tumors associated with Peutz-Jeghers syndrome.