纵隔生殖细胞肿瘤并发恶性血液病3例并文献复习

Three cases of mediastinal germ cell tumors and concurrent hematologic malignancy and literature review

总结纵隔生殖细胞肿瘤(GCTs)并发恶性血液病(HM)患儿的临床特征及治疗预后。回顾性收集2014年11月至2018年9月北京儿童医院肿瘤内科收治的3例纵隔GCTs并发HM患儿的临床资料,检索截至2019年12月PubMed数据库及万方数据库的相关文献并进行文献复习。3例患儿为12~16岁男童,以纵隔占位起病,GCTs病理类型为畸胎瘤或卵黄囊瘤,予手术和化疗治疗。3例在GCTs诊断的5个月、9个月、31个月时分别发生了急性髓细胞白血病(AML)、骨髓增生异常综合征,予对症治疗,2例死亡,1例存活。共检索到英文报道病例135例,其中纵隔GCTs并发HM共127例(94.1%),其他部位GCTs并发HM共8例(5.9%)。文献报道的127例纵隔GCTs并发HM病例中,126例(99.2%)为男性,诊断GCTs的中位年龄为22岁(10~48岁);GCTs病理类型包括畸胎瘤53例(41.7%),含畸胎瘤和/或卵黄囊瘤成分的GCTs共94例(74.0%);HM类型中,AML共72例(56.7%),其中AML-M7共31例(24.4%);GCTs与HM间隔中位时间为3个月(0~122个月),46例(36.2%)患儿2种疾病同时发生,85例(66.9%)在GCTs诊断的12个月内发生HM;可知生存状态的病例107例,其中存活13例(12.1%),死亡94例(87.9%),诊断HM后中位生存时间为2个月(0~48个月)。对于原发纵隔GCTs,尤其病理类型为卵黄囊瘤和/或畸胎瘤成分的青少年男性,需高度关注其HM倾向,此类患者预后极差,异基因造血干细胞移植是可选择的治疗方法。

To analyze the clinical characteristics,treatment and prognosis of mediastinal germ cell tumors(GCTs)with concurrent hematologic malignancy(HM).The clinical features,treatment and prognosis of 3 cases of HM associated with mediastinal GCTs treated in the Department of Medical Oncology,Beijing Children′s Hospital from November 2014 to September 2018 were retrospectively analyzed.Meanwhile,relevant cases were searched in the PubMed and Wanfang database from their establishment to December 2019.Three male cases of HM associated with mediastinal GCTs aged from 12 to 16 years.The pathogenesis of mediastinal masses suggested teratoma or yolk sac tumor.All of them were treated with surgery and chemotherapy.Acute myeloid leukemia(AML)or myelodysplastic syndromes(MDS)was diagnosed respectively at 5 months,9 months and 31 months after initial GCTs in the 3 cases.Two patients died and 1 child survived at the last follow-up.A total of 135 cases of concurrent GCTs and HM(or leukemia)were reviewed in online databases,involving 127 cases(94.1%)with the mediastinal GCTs associated with HM and 8 cases(5.9%)with GCTs related HM from another original sites.One hundred and twenty-six cases(99.2%)were male and the median age of GCTs diagnosis was 22(10-48)years.Fifty-three cases(41.7%)were teratoma and 94 cases(74.0%)were GCTs containing teratoma with or without yolk sac tumor.Among the types of HM,72 cases(56.7%)were AML and 31 cases(24.4%)were AML-M7.The median interval between GCTs and HM was 3(0-122)months.Forty-six cases(36.2%)presented 2 malignancies simultaneously.HM were diagnosed within 12 months of GCTs in 85 cases(66.9%).The survival data were known in 107 cases,involving 94(87.9%)deaths and 13(12.1%)survivors.The median survival time after diagnosis of HM was 2(0-48)months.The tendency of HM must be highly concerned in adolescent male patients with primary mediastinal GCTs,especially those with yolk sac tumor or teratoma.Their prognoses are very poor.Allogeneic hematopoietic stem cell transplantation is an alternative treatment.