儿童恶性生殖细胞肿瘤：意大利首个合作研究“TCG 91”的结果

Malignant germ cell tumors in childhood: results of the first Italian cooperative study "TCG 91".

作者信息

Lo Curto Margherita, Lumia Francesca, Alaggio Rita, Cecchetto Giovanni, Almasio Piero, Indolfi Paolo, Siracusa Fortunato, Bagnulo Serenella, De Bernardi Bruno, De Laurentis Tina, Di Cataldo Andrea, Tamaro Paolo

机构信息

Dipartimento Materno Infantile, University of Palermo, Italy.

出版信息

Med Pediatr Oncol. 2003 Nov;41(5):417-25. doi: 10.1002/mpo.10324.

DOI:10.1002/mpo.10324

PMID:14515380

Abstract

BACKGROUND AND AIMS

About 20% of patients with germ cell tumor (GCT) are still resistant to therapy. To investigate which features are present in resistant patients, a multicenter study on GCT in children was undertaken to correlate clinical and laboratory parameters with the outcome.

METHODS

Patients aged less than 16 years, with histologically proven extracranial GCT were included.

RESULTS

Ninety-five patients (median age 33 months, 45 males) were eligible. The site of the primary tumor was gonadal in 59, extragonadal in 36. The stage was I in 39; II in 5; IIIa (microscopic residue) in 7; IIIb (macroscopic residue) in 16; IIIc (unresectable) in 13; IV in 15. The treatment was surgery alone in 31; surgery plus radiotherapy in 1; chemotherapy +/- surgery in 63. Post-chemotherapy resection in 19 (10 complete, 9 partial). The chemotherapy regimen was carboplatin 400 mg/m2/day on days 1, 2; etoposide 150 mg/m2/day on days 1, 2; ifosfamide 1,500 mg/m2/day on days 21, 22; dactinomycin 1.5 mg/m2/day on day 21; vincristine 1.5 mg/m2/day on day 21. Three patients died because of toxicity and two non-responders (to primary chemotherapy), died of progression; among the remaining 90 patients 20 relapsed, 9 are in second remission, 2 are alive with disease, and 9 died of disease progression (one from progression and intracranial hemorrhage). Overall survival was 82.7% and event-free survival: 71.5%. Survival according to: (a) site: testis: 100%; ovary: 88%; sacrococcyx: 69.6%; other sites: 33.3% (P < 0.001); (b) stage: I and II: 100%; IIIa: 83.3%; IIIb: 84.6%; IIIc: 60.6%; IV: 53.2% (P < 0.001); (c) AFP levels: normal: 85.5%; 42-9,470 ng/ml: 84.6%; >/=10,000 ng/ml: 58.7% (P = 0.02). All the pts who had complete resection of the primary tumor at diagnosis or at delayed surgery, remained in remission.

CONCLUSIONS

Multivariate analysis showed that the primary site of tumor was the only independent prognostic factor for survival and EFS.

摘要

背景与目的

约20%的生殖细胞肿瘤（GCT）患者对治疗仍有抵抗性。为了研究抵抗性患者具有哪些特征，开展了一项关于儿童GCT的多中心研究，以将临床和实验室参数与治疗结果相关联。

方法

纳入年龄小于16岁、经组织学证实为颅外GCT的患者。

结果

95例患者（中位年龄33个月，45例男性）符合条件。原发肿瘤部位为性腺的有59例，性腺外的有36例。分期为I期的有39例；II期的有5例；IIIa期（微小残留）的有7例；IIIb期（肉眼可见残留）的有16例；IIIc期（无法切除）的有13例；IV期的有15例。治疗方式为单纯手术的有31例；手术加放疗的有1例；化疗±手术的有63例。19例（10例完全切除，9例部分切除）患者在化疗后进行了手术切除。化疗方案为：第1、2天卡铂400mg/m²/天；第1、2天依托泊苷150mg/m²/天；第21、22天异环磷酰胺1500mg/m²/天；第21天放线菌素D 1.5mg/m²/天；第21天长春新碱1.5mg/m²/天。3例患者因毒性死亡，2例（对初始化疗无反应）患者因病情进展死亡；在其余90例患者中，20例复发，9例处于第二次缓解期，2例带瘤生存，9例因疾病进展死亡（1例因进展和颅内出血）。总生存率为82.7%，无事件生存率为71.5%。生存率根据以下因素划分：（a）部位：睾丸：100%；卵巢：88%；骶尾骨：69.6%；其他部位：33.3%（P<0.001）；（b）分期：I期和II期：100%；IIIa期：83.3%；IIIb期：84.6%；IIIc期：60.6%；IV期：53.2%（P<0.001）；（c）甲胎蛋白（AFP）水平：正常：85.5%；42 - 9470ng/ml：84.6%；≥10000ng/ml：58.7%（P = 0.02）。所有在诊断时或延迟手术时对原发肿瘤进行了完全切除的患者均保持缓解状态。