18例原发性纵隔卵黄囊瘤的临床诊治及预后特点  

Diagnosis,treatment and prognosis of primary mediastinal yolk sac tumor in 18 patients

作　　者：高雪涵 陈野野[1] 张家齐[1] 李单青 GAO Xuehan;CHENYeye;ZHANG Jiaqi;LI Shanqing(Department of Thoracic Surgery,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing,100730,P.R.China)

机构地区：[1]中国医学科学院北京协和医学院北京协和医院胸外科,北京100730

出　　处：《中国胸心血管外科临床杂志》2023年第9期1280-1289,共10页Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

基　　金：国家重点研发计划(2020YFB1313700)。

摘　　要：目的探讨原发性纵隔卵黄囊瘤(primary mediastinal yolk sac tumor,PMYST)的临床特点及诊治经验。方法收集北京协和医院胸外科2000年9月—2020年9月收治的PMYST患者的临床资料。对其临床特点、病理学特征、治疗方式及预后情况进行回顾性分析。结果纳入18例患者,其中男17例、女1例,中位年龄22.0(16.6,26.2)岁。患者伴有甲胎蛋白水平升高,病理类型可为单一卵黄囊瘤或与其他纵隔生殖细胞肿瘤合并存在。治疗方式以化疗及根治性手术为主,对肿瘤侵犯其他脏器的患者可行扩大切除。手术患者7例术后出现肺或胸膜转移,其中3例伴有胸腔外(骨、脑)转移;1例术后1年内出现复发。所有患者均通过电话或门诊随访,末次随访时5例生存,9例死亡,4例失访,中位生存期为16.8个月;中位无进展生存期为14.9个月;术后1年、3年、5年生存率分别为73.3%、28.6%和11.8%。结论PMYST常发生于中青年男性患者,术前穿刺能提供有效的诊断依据。R0切除、治疗后甲胎蛋白恢复正常、不伴有转移可能是预后较好的潜在指标。PMYST总体预后较差,经合理诊治后部分患者可获得长期生存。Objectivee To investigate the clinical characteristics and treatment of primary mediastinal yolk sac tumor(PMYST).Methods We collected the clinical data of PMYST patients who were admitted to the Department of Thoracic Surgery of Peking Union Medical College Hospital from September 2000 to September 2020.The clinical and pathological characteristics,treatment and prognosis of PMYST patients were retrospectively analyzed.ResultsFinally 18 patients were enrolled,including 17 males and 1 female with a median age of 22.0(16.6,26.2)years.Patients had increased level of alpha fetoprotein(AFP).The pathological type can be single yolk sac tumor or combined with other mediastinal germ cell tumors.Chemotherapy and radical surgery were the main treatment methods.Extensive resection was feasible for patients with tumor invasion of other organs.Seven patients developed lung or pleural metastasis after operation,and 3 of them had extrapleural metastasis.One patient recurred within 1 year after surgery.All patients were followed up by telephone or outpatient department.At the last follow-up,5 patients survived,9 died,and 4 were lost to follow up,with a median survival of 16.8 months.The median disease-free survival was 14.9 months.The survival rates at 1,3 and 5 years were 73.3%,28.6%and 11.8%,respectively.Conclusion PMYST often occurs in young-middle aged male patients.Preoperative puncture can provide an effective diagnostic basis.RO resection,AFP returning to normal after treatment and no metastasis may be potential indicators of good prognosis.The overall prognosis of PMYST is poor,and some patients can achieve long-term survival after treatment.

关 键 词：原发性纵隔卵黄囊瘤 内胚窦瘤 病理学特征 预后 

分 类 号：R734.5[医药卫生—肿瘤]