儿童颅外恶性生殖细胞肿瘤的临床特点及预后分析

Clinical analysis and prognosis of children with extracranial malignant germ cell tumors

目的:总结儿童颅外恶性生殖细胞肿瘤的临床特点及影响预后的因素。方法:回顾性分析2015年01月至2019年12月我院收治的49例颅外恶性生殖细胞肿瘤的临床资料。结果:共入组49例颅外恶性生殖细胞肿瘤患者,男性29例,女性20例,男女比例1.45∶1;中位发病年龄20月(4~189月);<4岁42例,4~11岁4例,>11岁3例;原发瘤灶位于性腺者30例(睾丸22例,卵巢8例),性腺外者19例(纵隔2例,腹腔1例,盆腔4例,阴道4例,骶尾部8例);Ⅰ期6例,Ⅱ期16例,Ⅲ期14例,Ⅳ期13例;低危组6例,中危组24例,高危组19例;病例类型:卵黄囊瘤31例,恶性畸胎瘤12例,混合型恶性生殖细胞肿瘤6例。所有患儿均行手术、化学治疗,部分患者行放疗。中位随访时间49月,经治疗全组CR 44例,PR 1例,复发或进展4例,其中死亡2例。2年总生存率(OS)和无事件生存率(EFS)分别为(98.0±2.0)%和(93.9±3.4)%。5年OS和EFS为(95.6±3.1)%和(91.7±4.0)%。结论:以铂类为主的化疗对于儿童颅外恶性生殖细胞肿瘤治疗效果肯定,初诊患者AFP≥10000 ng/mL及年龄>11岁与预后不良相关,复发/难治患者可行常规二线化疗、大剂量化疗、自体干细胞移植、放疗等综合治疗。

Objective:To summarize the clinical features and prognosis of extracranial malignant germ cell tumor in children.Methods:A retrospective analysis was performed on 49 cases with extracranial malignant germ cell tumor in our hospital from January 2015 to December 2019.Results:Totally 49 patients were included in the study, including 29 males and 20 females, with a median age of 20 months.42 cases were <4 years old, 4 cases were between 4 and 11 years old, 3 cases were >11 years old.The primary tumor were located at gonad in 30 cases(22 cases in testis, 8 cases in ovary) and extragonadal sites in 19 cases(2 cases in mediastinum, 1 case in enterocoelia, 4 cases in pelvic cavity, 4 cases in vagina, and 8 cases in sacrococcygeal region).6 cases were in stage Ⅰ,16 cases in stage Ⅱ,14 cases in stage Ⅲ,and 13 cases in stage Ⅳ.6 cases were at low risk, 24 cases at intermediate risk, and 19 cases at high risk.31 cases were yolk sac tumor, 12 cases were malignant teratoma, and 6 cases were mixed germ cell tumor.All patients were treated with chemotherapy and surgical treatment, some with radiotherapy.The median follow-up time was 49 months, complete remission in 44 cases, partial response in 1 case, recurrence or progression in 4 cases.The 2-year OS rate and EFS rate were(98.0±2.0)% and(93.9±3.4)%.The 5-year OS rate and EFS rate were(95.6±3.1)% and(91.7±4.0)%,respectively.Conclusion:Chemotherapy with platinum-based obtained a positive effect in children with extracranial MGCT.AFP≥10 000 ng/mL,age>11 years old were the factors of poor prognosis patients with tumor.Progression can be treated by second-line conventional chemotherapy(SLCT),high dose of chemotherapy(HDCT),autologous stem cell transplantation(ASCT) and radiotherapy.