原发性纵隔卵黄囊瘤6例临床与病理特点  

Clinical and pathological characteristics of 6 cases of primary mediastinal yolk sac tumor

作　　者：薛治乾 王名法 何圣科 冯海玲 陈辉 XUE Zhiqian;WANG Mingfa;HE Shengke;FENG Hailing;CHEN Hui(Department of Pathology,Danzhou People’s Hospital,Danzhou Hainan 571700;Department of Pathology,Second Affiliated Hospital of Hainan Medical University,Haikou 570100;Department of Pathology,Hainan Cancer Hospital,Haikou 570100,China)

机构地区：[1]儋州市人民医院病理科,海南儋州571700 [2]海南医科大学第二附属医院病理科,海口570100 [3]海南省肿瘤医院病理科,海口570100

出　　处：《临床与病理杂志》2024年第12期1712-1719,共8页Journal of Clinical and Pathological Research

摘　　要：目的:原发性纵隔卵黄囊瘤(primary mediastinal yolk sac tumor,PMYST)罕见,具有恶性程度高、病情发展快及预后较差的特点。本研究探讨PMYST的临床病理特征、诊断及鉴别诊断、治疗及预后。方法:回顾性分析6例PMYST的临床病理学形态、免疫表型及肿瘤生物学行为,并复习相关文献。结果:6例患者均为男性,发病年龄15~53(平均30)岁,血清甲胎蛋白(alpha fetoprotein,AFP)水平均异常升高,胸部CT显示前中、前右及前纵隔软组织肿块,肿块最大径6~17(平均12) cm。病理镜下细胞呈疏松网状、乳头状或腺管状,可见典型Schiller-Duval(S-D)小体。免疫表型显示:6例AFP、婆罗双树样基因4(spalt like transcription factor 4,SALL4)、前列腺酸性磷酸酶(placental alkaline phosphatase,PLAP)、广谱细胞角蛋白(pan-cytokeratin,CK-pan)、Glypican-3均阳性,其中1例CKpan及1例AFP部分阳性,3例PLAP灶状阳性。1例波形蛋白阳性,1例CD117弱阳性,CD30、八聚体结合转录因子3/4(octamer-binding transcription factor 3/4,OCT3/4)及上皮膜抗原(epithelial membrane antigen,EMA)均阴性,Ki-67增殖指数为80%~90%。4例接受穿刺活检,其中1例治疗后手术切除肿块;另2例直接手术切除肿块。6例均诊断为卵黄囊瘤,其中2例合并精原细胞瘤。6例均接受BEP(博来霉素+依托泊苷+顺铂)或EP(依托泊苷+顺铂)方案化疗,其中2例联合放疗。6例随访7~36个月,4例死亡,1例出现肺转移,1例未出现转移及复发。结论:PMYST多发于青年男性患者,恶性程度较高,生存率偏低;影像学检查提示纵隔肿块且AFP异常升高有助于诊断,病理检查可确诊;术后以BEP或EP方案化疗为主,血清AFP值可作为预后监测指标。Objective:Primary mediastinal yolk sac tumor(PMYST)is a rare malignancy characterized by high-grade malignancy,rapid progression,and poor prognosis.This study aims to explore the clinical and pathological characteristics,diagnostic criteria,differential diagnosis,treatment strategies,and prognosis of primary mediastinal yolk sac tumor(PMYST).Methods:A retrospective analysis was conducted on the clinical,pathological,immunophenotypic characteristics,and morphology,immunophenotype,and tumor biological behavior of 6 cases of PMYST,accompanied by a literature review.Results:All 6 patients were male,with an onset aged 15 to 53 years(average age:30 years).Serum alpha-fetoprotein(AFP)levels were significantly elevated in all cases.Chest CT scans revealed anterior-middle,anterior-right,or anterior mediastinal soft tissue masses,with a maximum tumor diameter ranging from 6 to 17 cm(mean:12 cm).Histopathology showed loose reticular,papillary,or glandular structures with characteristic Schiller-Duval bodies.Immunohistochemistry revealed positivity for AFP,spalt-like transcription factor 4(SALL4),pan-cytokeratin(CK-pan),placental alkaline phosphatase(PLAP),and Glypican-3 in all 6 cases,with focal positivity for CK-pan and AFP in 1 case each,and focal PLAP positivity in 3 cases.One case was positive for vimentin,and 1 showed weak CD117 positivity.All cases were negative for CD30,octamer-binding transcription factor 3/4(OCT3/4),and epithelial membrane antigen(EMA).The Ki-67 proliferation index ranged from 80%to 90%.Among the 6 cases,4 underwent biopsy,with 1 subsequently receiving surgical resection post-treatment,and 2 underwent direct surgical resection.All 6 cases were diagnosed as yolk sac tumors,with 2 cases showing mixed seminoma components.All patients received chemotherapy(BEP or EP regimens),and 2 also underwent radiotherapy.Follow-up ranged from 7 to 36 months:Four patients died,1developed pulmonary metastasis,and 1 showed no recurrence or metastasis.Conclusion:PMYST predominantly affects young male patients and is h

关 键 词：卵黄囊瘤 纵隔 青年男性 病理学特征 临床表现 

分 类 号：R734.5[医药卫生—肿瘤]