根据自然病史和手术治疗情况预测恶性骶尾部生殖细胞肿瘤的预后。

Prognosis of malignant sacrococcygeal germ cell tumours according to their natural history and surgical management.

机构信息

Paediatric Surgical Unit, Padua, Italy.

出版信息

Surg Oncol. 2012 Jun;21(2):e31-7. doi: 10.1016/j.suronc.2012.03.001. Epub 2012 Mar 28.

DOI:10.1016/j.suronc.2012.03.001

PMID:22459912

Abstract

INTRODUCTION

Malignant sacrococcygeal (SC) germ cell tumours (GCT) may be diagnosed as primary pelvic tumour or malignant recurrence of foetal SC teratoma (FSCT) operated during the neonatal period. In order to evaluate the difference between these two populations, the authors report their experience with SC-GCT registered in the French TGM 95 protocol.

POPULATION AND METHODS

The protocol comprised risk-adapted-chemotherapy (CT) followed by surgery. Standard risk (SR: localized tumour completely resected) had no adjuvant therapy. Intermediate-Risk (IR: localized tumour, incomplete or no initial surgery with αFP<15,000 ng/ml) received Vinblastine-Bleomycin-Cisplatin regimen; while High-Risk (HR: αFP > 15,000 ng/ml and/or metastases) received Etoposide-Ifosfamide-Cisplatin.

RESULTS

Fifty-seven patients with SC-GCT, aged 0-80 months (median 16), were registered between 1995 and 2005. Nineteen patients had secondary SC-GCT after FSCT. All patients received CT: 17 IR and 1 SR after reevolution; 39 HR (25 with metastases). 51 patients underwent delayed surgery, which was incomplete in 8 patients.

EVOLUTION

Seventy-two percent of the secondary SC-GCT had systematic biological follow-up. αFP increasing was the first presenting sign in 80% of the cases. Patients with secondary SC-GCT had a lower median αFP level at diagnosis, were less frequently classified as HR and received less CT. The two groups with secondary vs. primary SC-GCT had a statistically similar favourable outcome (Overall Survival: 93.8% vs. 86.2%; Event-Free Survival: 89.2 vs. 78.2%; p > 0.34 and >0.32), respectively, but with less burden of therapy.

CONCLUSIONS

SC-GCT has a good overall prognosis provided complete surgery is achieved and CT is administered to IR and HR patients. SC-GCT in patients followed by αFP after treatment for FSCT had less tumour extension than newly-diagnosed patients, probably because of earlier detection of the disease.

摘要

介绍

恶性尾骨（SC）生殖细胞瘤（GCT）可被诊断为原发性盆腔肿瘤或新生儿期手术切除的胎儿 SC 畸胎瘤（FSCT）的恶性复发。为了评估这两个群体之间的差异，作者报告了他们在法国 TGM 95 方案中登记的 SC-GCT 的经验。

人群和方法

该方案包括风险适应化疗（CT）后手术。标准风险（SR：完全切除的局部肿瘤）无辅助治疗。中危（IR：局部肿瘤，初始手术不完全或无手术，αFP<15000ng/ml）接受长春碱-博来霉素-顺铂方案；高危（HR：αFP>15000ng/ml 和/或转移）接受依托泊苷-异环磷酰胺-顺铂。

结果

1995 年至 2005 年间，57 例 SC-GCT 患者（年龄 0-80 个月，中位数 16 岁）登记。19 例为 FSCT 后继发性 SC-GCT。所有患者均接受 CT：17 例 IR 和 1 例 SR 后复发；39 例 HR（25 例有转移）。51 例患者接受了延迟手术，其中 8 例手术不完全。

演变

72%的继发性 SC-GCT 进行了系统的生物学随访。80%的病例以αFP 升高为首发表现。继发性 SC-GCT 患者诊断时的中位αFP 水平较低，HR 分类较少，接受的 CT 较少。继发性和原发性 SC-GCT 两组的总体生存（93.8% vs. 86.2%）和无事件生存（89.2% vs. 78.2%）均有统计学上相似的良好结果（p>0.34 和>0.32），但治疗负担较小。