儿童和青少年原发性纵隔生殖细胞肿瘤：德国合作方案MAKEI 83/86、89和96的结果

Primary mediastinal germ cell tumors in children and adolescents: results of the German cooperative protocols MAKEI 83/86, 89, and 96.

作者信息

Schneider D T, Calaminus G, Reinhard H, Gutjahr P, Kremens B, Harms D, Göbel U

机构信息

Department of Pediatric Hematology and Oncology, Heinrich-Heine-University, D]usseldorf, Germany.

出版信息

J Clin Oncol. 2000 Feb;18(4):832-9. doi: 10.1200/JCO.2000.18.4.832.

DOI:10.1200/JCO.2000.18.4.832

PMID:10673525

Abstract

PURPOSE

To evaluate children and adolescents with primary mediastinal teratoma and malignant germ cell tumors (GCTs).

PATIENTS AND METHODS

Forty-seven patients from the German nontesticular GCT studies were analyzed (median age, 2.5 years; range, neonate to 17 years). Teratoma (n = 21) were resected, and no adjuvant treatment was given. Malignant GCTs (n = 26) were treated with cisplatin-based chemotherapy and resection. Three of 26 patients underwent radiotherapy.

RESULTS

In all patients with teratoma, tumor markers were normal. Surgery of teratoma was complete in 17 of 21 patients and microscopically incomplete in four of 21 patients, and we observed no relapse after a median follow-up of 29 months. In 23 of 26 patients with malignant GCTs, alpha-fetoprotein and/or beta-human chorionic gonadotropin were elevated. Twelve of 26 patients received adjuvant chemotherapy after initial resection, which was complete in six of 12 patients, whereas delayed resection after preoperative chemotherapy was complete in 10 of 11 patients (P =.03). Four of six patients underwent second-look thoracotomy after incomplete primary surgery. Three of 26 patients did not undergo tumor resection. The final completeness of resection was the strongest prognostic indicator (event-free survival ¿EFS, 0.94 +/- 0.06 v 0.42 +/- 0.33; P <.002). Local stage and distant metastases were not prognostically significant at the.05 level. For all malignant GCTs, the 5-year survival rate was 0.87 +/- 0.05 (median follow-up, 51 months), with an EFS of 0.83 +/- 0.05.

CONCLUSION

The prognosis of mediastinal teratoma is excellent after complete or microscopically incomplete resection. In children with malignant GCT, the prognosis is favorable with a therapeutic strategy of delayed resection after preoperative chemotherapy. In most children, the diagnosis can be based on elevated tumor markers and imaging. Biopsy is indicated in nonsecreting GCT.

摘要

目的

评估患有原发性纵隔畸胎瘤和恶性生殖细胞肿瘤（GCTs）的儿童及青少年。

患者与方法

对来自德国非睾丸GCT研究的47例患者进行分析（中位年龄2.5岁；范围，新生儿至17岁）。21例畸胎瘤患者接受了手术切除，未给予辅助治疗。26例恶性GCT患者接受了以顺铂为基础的化疗及手术切除。26例患者中有3例接受了放疗。

结果

所有畸胎瘤患者的肿瘤标志物均正常。21例畸胎瘤患者中17例手术完整，4例显微镜下不完整，中位随访29个月后未观察到复发。26例恶性GCT患者中有23例甲胎蛋白和/或β-人绒毛膜促性腺激素升高。26例患者中有12例在初次切除后接受了辅助化疗，其中12例中有6例手术完整，而术前化疗后延迟切除的11例患者中有10例手术完整（P = 0.03）。6例初次手术不完整的患者中有4例接受了二次开胸探查。26例患者中有3例未接受肿瘤切除。最终切除的完整性是最强的预后指标（无事件生存率[EFS]，0.94±0.06对0.42±0.33；P<0.002）。局部分期和远处转移在0.05水平上无预后意义。所有恶性GCT患者的5年生存率为0.87±0.05（中位随访51个月），EFS为0.83±0.05。