Lack E E, Weinstein H J, Welch K J
J Thorac Cardiovasc Surg. 1985 Jun;89(6):826-35.
Twenty-one patients aged 16 years or less had been treated for a primary mediastinal germ cell tumor at the Children's Hospital, Boston Massachusetts, during the last 54 years. There were 13 boys and eight girls with the average age at diagnosis being 7 years (range 2 weeks to 16 years). Twelve mediastinal germ cell tumors were classified as pure teratoma, five contained embryonal carcinoma admixed with other germ cell components, and four were pure embryonal carcinoma. Of 12 patients with pure teratoma, 10 underwent complete surgical resection and were alive and well 1 to 13 years later; two children left untreated died of complications related to local tumor growth. Complete surgical resection was possible for only two of nine patients with embryonal carcinoma; both received adjuvant therapy and were alive and well 3 and 20 years later. Seven patients received radiation and/or chemotherapy but died of residual or metastatic disease. Successful treatment for children with embryonal carcinoma requires an operation aimed at either debulking or complete resection (if possible) coupled with early and aggressive combination chemotherapy. The role of radiation in primary therapy remains undefined with regard to curative intent.
在过去54年里,马萨诸塞州波士顿儿童医院对21名16岁及以下的儿童进行了原发性纵隔生殖细胞肿瘤的治疗。其中有13名男孩和8名女孩,诊断时的平均年龄为7岁(范围为2周龄至16岁)。12例纵隔生殖细胞肿瘤被分类为纯畸胎瘤,5例含有胚胎癌并混有其他生殖细胞成分,4例为纯胚胎癌。12例纯畸胎瘤患者中,10例接受了完整的手术切除,术后1至13年仍存活且状况良好;2例未接受治疗的儿童死于与局部肿瘤生长相关的并发症。9例胚胎癌患者中只有2例可行完整的手术切除;这2例均接受了辅助治疗,术后3年和20年仍存活且状况良好。7例患者接受了放疗和/或化疗,但死于残留或转移性疾病。对胚胎癌患儿进行成功治疗需要进行减瘤手术或完整切除手术(若可能),并结合早期积极的联合化疗。就治愈目的而言,放疗在初始治疗中的作用仍不明确。