目的 总结女性生殖系统中恶性中胚叶混合瘤（MMMT）的临床病理特征及预后，分析P53及错配修复蛋白与MMMT发病之间的关系。方法 收集大理大学第一附属医院2015年9月—2022年9月15例经手术切除病理诊断为MMMT的病例，总结临床病理特点、免疫表型（P53、错配修复蛋白等）、治疗方案并随访。结果 15例MMMT原发于子宫10例，卵巢5例。发病年龄范围49~76岁，平均年龄60岁，中位年龄58岁。临床表现为阴道流血或流液，伴或不伴腹痛或盆腔包块。镜下肿瘤均由不同比例的恶性上皮和间叶源性肿瘤构成，P53野生型12例，突变型3例；错配修复蛋白（MSH6、MSH2、MLH1、PMS2）检测存在缺失的有4例。15例患者中均行手术治疗，12例行盆腔淋巴结清扫术，术后辅以放化疗。随访失访2例，死亡4例，复发6例，3例术后无复发和转移。结论 恶性中胚叶混合瘤临床少见，恶性程度高，病理诊断上存在困难，需要辅以免疫组织化学染色，P53及错配修复蛋白缺失与MMMT的发生存在一定关系。治疗上需要手术切除，辅以放化疗。
Objective To summarize the clinical and pathological characteristics and prognosis of malignant mesodermal mixed tumor（MMMT）in the female reproductive system，and analyze the relationship between P53 and mismatch repair proteins and the onset of MMMT．Methods A total of 15 cases diagnosed with MMMT after surgical resection at the First Affiliated Hospital of Dali University from September 2015 to September 2022 were collected．The clinical and pathological characteristics，immune phenotype（P53，mismatch repair protein，etc. ），treatment plan were summarized．And the patients were followed-up．Results Ten of 15 cases of MMMT were primary in the uterus and 5 of 10 in the ovaries．The age range of onset was 49 to 76 years old，with an average age of 60 and a median age of 58．Clinical manifestations included vaginal bleeding or fluid discharge，with or without abdominal pain or pelvic masses．Under the microscope，all tumors were composed of malignant epithelial and mesenchymal tumors in different proportions，with 12 cases of P53 wild-type and 3 cases of mutant type．There were 4 cases of missing mismatch repair proteins（MSH6，MSH2，MLH1，PMS2）detected．Among the 15 patients，all underwent surgical treatment，and 12 underwent pelvic lymph node dissection with postoperative adjuvant chemotherapy and radiotherapy．Two cases were lost to follow-up，four cases died，six cases recurred，and three cases had no recurrence or metastasis after surgery．Conclusions MMMT are rare in clinical practice，with high malignancy and poor prognosis．Pathological diagnosis is difficult，and immunohistochemical staining is needed．The absence of P53 and mismatch repair protein is related to the occurrence of MMMT. Surgical resection is required for treatment，supplemented by radiotherapy and chemotherapy．
