Lu L H, Chen Y Q, Li J, Shao S S, Ma F H, Ning Y, Shi Y, Wang C
Department of Pathology, Obstetrics and Gynecology Hospital of Fudan University, Shanghai200090, China.
School of Clinical Medicine, Shanghai Medical College, Fudan University, Shanghai200032, China.
Zhonghua Zhong Liu Za Zhi. 2024 Dec 23;46(12):1195-1208. doi: 10.3760/cma.j.cn112152-20240518-00201.
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. 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. 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 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 . 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 (＜0.05). 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 cervix may have a better prognosis than gastric-type mucinous adenocarcinoma of the cervix alone, so the accurate diagnosis of SMMN-FGT is critical for clinical management.
女性生殖道同期黏液化生和肿瘤形成（SMMN - FGT）在同一时期发生于多个部位极为罕见，本研究旨在探讨SMMN - FGT的临床病理特征及其与预后的关系。我们回顾性分析了2012年1月至2022年10月在复旦大学附属妇产科医院病例数据库中诊断的25例SMMN - FGT患者的临床病理特征及随访记录。发病年龄中位数分别为46岁。临床表现包括不规则阴道出血或排液、盆腔疼痛和卵巢囊肿等。胚系基因检测在2例患者中确诊为黑斑息肉综合征（P - J综合征）。所有患者均接受了手术，13例患者术后接受了辅助放疗和/或化疗。最常见的病变部位是宫颈（21例），子宫内膜、输卵管和卵巢分别有11例、10例和16例。6例同时累及三个部位，仅1例同时累及所有四个部位。在9例具有突变表型的病例中，6例为胃型黏液腺癌，2例为宫颈小叶状腺上皮增生，1例为黏液腺癌，而所有微偏离腺癌均具有野生型表型。中位随访时间为59个月，期间3例死亡，6例出现局部复发或远处转移。根据我们的分析，术后复发或转移与疾病的FIGO分期、病变部位数量以及子宫病变严重程度相关（＜0.05）。SMMN - FGT具有相对较好的临床预后，即使是晚期患者也可从手术和辅助治疗中获益。对于年轻患者，如果经过充分评估未发现病变证据，可保留卵巢。在SMMN - FGT中，宫颈发生的胃型黏液腺癌可能比单纯宫颈胃型黏液腺癌预后更好，因此准确诊断SMMN - FGT对临床管理至关重要。
