女性生殖道同期多部位发生的黏液上皮化生和肿瘤的临床病理特征及预后分析  

Clinicopathological features and prognostic analysis of synchronous mucinous metaplasia and neoplasia of the female genital tract

作　　者：陆铃慧 陈奕清 李静 邵思齐 马凤华[3] 宁燕 石月 王超 Lu Linghui;Chen Yiqing;Li Jing;Shao Siqi;Ma Fenghua;Ning Yan;Shi Yue;Wang Chao(Department of Pathology,Obstetrics and Gynecology Hospital of Fudan University,Shanghai 200090,China;School of Clinical Medicine,Shanghai Medical College,Fudan University,Shanghai 200032,China;Department of Radiology,Obstetrics and Gynecology Hospital of Fudan University,Shanghai 200090,China;Department of Gynaecology and Obstetrics,Obstetrics and Gynecology Hospital of Fudan University,Shanghai 200090,China;Department of Gynecological Oncology,Obstetrics and Gynecology Hospital of Fudan University,Shanghai 200090,China)

机构地区：[1]复旦大学附属妇产科医院病理科,上海200090 [2]复旦大学上海医学院临床医学院,上海200032 [3]复旦大学附属妇产科医院影像科,上海200090 [4]复旦大学附属妇产科医院妇产科,上海200090 [5]复旦大学附属妇产科医院妇科肿瘤科,上海200090

出　　处：《中华肿瘤杂志》2024年第12期1195-1208,共14页Chinese Journal of Oncology

基　　金：上海市自然基金项目(22ZR1408900);上海市申康临床三年行动计划青年项目(SHDC2020CR4079);国家自然科学基金面上项目(82273233、81772777);上海市“医苑新星”杰出青年医学人才项目(SHWJRS2021-99)。

摘　　要：目的探讨女性生殖道同期多部位发生的黏液上皮化生和肿瘤(SMMN-FGT)的临床病理特征及其预后。方法回顾性分析复旦大学附属妇产科医院自2012年1月至2022年12月诊断的25例SMMN-FGT患者的临床病理特征及随访记录。生存分析采用Kaplan-Meier法和Log rank检验。结果25例患者的发病中位年龄为46岁,临床表现包括阴道不规则流血或排液、盆腔疼痛、卵巢囊肿等。2例患者胚系检测证实为Peutz-Jeghers综合征。所有患者均接受手术治疗,13例患者术后辅助放疗和/或化疗。最常发生病变的部位是宫颈(21例),发生在子宫内膜、输卵管及卵巢的病例数分别为11例、10例及16例。25例患者中,6例同时累及3个部位,仅1例出现4个部位同时受累。9例P53突变表型中,胃型腺癌6例,宫颈叶状增生2例,黏液腺癌1例,所有微偏腺癌P53均呈野生表型。中位随访时间为59个月,死亡3例,6例出现局部复发或远处转移。术后是否复发转移与疾病国际妇产科联盟分期、病变部位的数目及子宫体病变的程度有关(均P<0.05)。结论SMMN-FGT临床预后相对较好,即使是晚期患者也能从手术及辅助治疗中获益。对于年轻患者,如卵巢经充分评估未见病变可予以保留。SMMN-FGT中宫颈发生胃型腺癌可能较单纯宫颈胃型腺癌预后良好,正确诊断SMMN-FGT对临床处理至关重要。Objective Synchronous mucinous metaplasia and neoplasia of the female genital tract(SMMN-FGT)occurring at multiple sites during the same period of time is extremely rare,and the aim of this study was to investigate the clinicopathologic features of SMMN-FGT and its relationship with prognosis.Methods We retrospectively analyzed the clinicopathological features and follow-up records of 25 cases of SMMN-FGT diagnosed from January 2012 to October 2022 in the case database of Obstetrics and Gynecology Hospital of Fudan University.Results The median age at onset was 46 years old,respectively.Clinical manifestations included irregular vaginal bleeding or drainage,pelvic pain,and ovarian cysts,etc.Germline genetic test confirmed Peutz-Jeghers syndrome(P-J syndrome)in two patients.All patients underwent surgery,and 13 patients had postoperative adjuvant radiotherapy and/or chemotherapy.The most frequent site of lesion was the cervix(21 cases),with 11,10 and 16 cases occurring in the endometrium,fallopian tubes and ovaries,respectively.Six cases involved three sites simultaneously,and only one case had all four sites involved at the same time.Among the 9 cases with P53 mutation phenotype,6 cases had gastric-type mucinous adenocarcinoma,2 cases had lobular endocervical glandular hyperplasia,and 1 case had mucinous adenocarcinoma,whereas all the minimally deviated adenocarcinomas had wild phenotype of P53.The median follow-up time was 59 months,during which 3 cases died and 6 cases developed local recurrence or distant metastasis.According to our analysis,postoperative recurrence or metastasis was correlated with the FIGO stage of the disease,the number of lesion sites and the severe degree of the uterine lesions(P<0.05).Conclusions SMMN-FGT has a relatively good clinical prognosis,and even advanced patients can benefit from surgery and adjuvant therapy.In young patients,the ovaries may be preserved if no evidence of lesions are seen after adequate evaluation.In SMMN-FGT,gastric-type mucinous adenocarcinoma occurring in the

关 键 词：女性生殖道肿瘤 黏液上皮化生和肿瘤 临床表现 病理特征 预后 

分 类 号：R737.3[医药卫生—肿瘤]