Liu Denghui,Li Yong,Li Ming,et al.Clinical diagnosis and treatment of children with extracranial mixed germ cell tumors:a report of 13 cases[J].Journal of Clinical Pediatric Surgery,2026,(05):467-471.[doi:10.3760/cma.j.cn101785-202504014]
儿童颅外混合性生殖细胞肿瘤诊治分析
- Title:
- Clinical diagnosis and treatment of children with extracranial mixed germ cell tumors:a report of 13 cases
- Keywords:
- Neoplasms; Germ Cell and Embryonal; Diagnosis; Treatment; Prognosis; Surgical Procedures; Operative; Child
- 摘要:
- 目的 初步探讨儿童颅外混合性生殖细胞肿瘤(mixed germ cell tumor,MGCT)的临床特征、诊断、治疗及预后。方法 回顾性分析2015年1月至2023年6月中南大学湘雅医学院附属儿童医院(湖南省儿童医院)普外科收治的13例颅外MGCT患儿临床及随访资料,总结颅外MGCT的临床特征、诊断治疗经过及预后情况。结果 13例中,男5例、女8例;发病年龄4天至12岁9个月,中位发病年龄10岁;原发瘤灶位于性腺7例(睾丸2例、卵巢5例),性腺外6例(纵隔3例、腹膜后2例、骶尾部1例)。参照儿童肿瘤协作组(Children’s Oncology Group,COG)标准分期:Ⅰ期3例,Ⅱ期2例,Ⅲ期3例,Ⅳ期5例;根据恶性生殖细胞国际联盟(Malignant Germ Cell International Alliance,MaGIC)危险度分组:低危组4例,中危组3例,高危组6例。13例初诊时甲胎蛋白(alpha fetoprotein,AFP)均有升高,7例初诊时β-人绒毛膜促性腺激素(β-human chorionic gonadotropin,β-HCG)升高。13例均接受手术联合化疗,其中12例完全缓解、1例复发,无一例死亡。中位随访时间33个月,均预后良好。结论 颅外MGCT需结合影像学、AFP及β-HCG检测进行诊断,手术联合放化疗可显著改善预后。
- Abstract:
- Objective Toexplore the clinical characteristics,diagnoses,treatments and prognoses of extracranial mixed germ cell tumor (MGCT) in children.Methods A retrospective analysis was performed on the clinical and follow-up data of 13 children with extracranial mixed germ cell tumors (MGCT) admitted to Affiliated Children’s Hospital,Xiangya School of Medicine,Central South University(Hunan Children’s Hospital),from January to June 2023.The clinical characteristics,diagnoses,treatments and prognoses of extracranial MGCT children were summarized.Results Among them,there were 5 boys and 8 girls with a median onset age of 10 year (4 day to 153 month).Primary tumors were located at gonad (n=7)(2 cases in testis,5 cases in ovary) and extra gonadal sites (n=6)(3 cases in mediastinum,2 in retroperitoneum,1 in sacrococcygeal region).According to the criteria of Children’s Oncology Group (COG),the clinical stages were Ⅰ (n=3),Ⅱ (n=2),Ⅲ (n=3) and Ⅳ (n=5).Additionally,according to the risk classification of Malignant Germ Cell International Alliance (MaGIC),the clinical risks were low (n=4),moderate (n=3) and high (n=6).Upon an initial diagnosis,all serum levels of alpha fetoprotein (AFP) were elevated while β-human chorionic gonadotropin (β-HCG) rose (n=7).All of them underwent surgery plus chemotherapy.The outcomes were complete remission (n=12) and recurrence (n=1).There was no death.The follow-up cutoff was June 2024 with a median follow-up of 33 month.Conclusion Extracranial MGCT diagnosis is dependent upon imaging and tumor markers (AFP/β-HCG).Surgical resection plus chemoradiotherapy may achieve a favorable prognosis.
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备注/Memo
收稿日期:2025-4-5。
基金项目:湖南省儿童实体瘤临床医学研究中心项目(2023SK4058)
通讯作者:李勇,Email:liyongpuwaike@163.com