胸内原始神经外胚层肿瘤的疗效和预后因素评估：84 例研究。

Evaluation of outcome and prognostic factors in thoracic primitive neuroectodermal tumor: a study of 84 cases.

机构信息

Department of Medical Oncology, Dr BR Ambedkar Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India.

出版信息

Ann Thorac Surg. 2013 Dec;96(6):2006-14. doi: 10.1016/j.athoracsur.2013.06.062. Epub 2013 Sep 12.

DOI:10.1016/j.athoracsur.2013.06.062

PMID:24035307

Abstract

BACKGROUND

Data on thoracic primitive neuroectodermal tumor (PNET) treated with a uniform chemotherapy protocol are minimal in the literature. We analyzed patients with thoracic PNET for outcome and prognostic factors.

METHODS

This is a single-institutional data review of patients treated between June 2003 and November 2011 with uniform neoadjuvant chemotherapy, surgical intervention, or radiotherapy (RT), or a combination of these treatments as local therapy followed by adjuvant chemotherapy.

RESULTS

Thoracic PNET was found in 84 of 374 (22%) patients with PNET with a median age of 15 years (range, 3-40 years); 27 (32%) of these patients had metastases. Thirty patients underwent surgical resection; 27 patients received radical RT after neoadjuvant chemotherapy. The radical RT group did not have adverse tumor characteristics or poor response to neoadjuvant chemotherapy. At median follow-up of 20.8 months (range, 2-104.6 months), 5-year event-free survival (EFS), overall survival (OS), and local control rate (LCR) were 24.4% ± 5.9%, 47.9% ± 8.4%, and 59.3% ± 9%, respectively, for the entire cohort, and 31% ± 7.7%, 59% ± 10.4%, and 67% ± 9.7%, respectively, for the group with localized tumors. In multivariate analysis, symptom duration longer than 4 months (p = 0.03), primary tumor of skeletal origin (p = 0.03), and radical RT (p = 0.006) predicted inferior EFS in the entire cohort and those with localized disease; metastatic disease (p = 0.002) predicted inferior OS. Radical RT predicted inferior LCR in the entire cohort and the group with localized tumor; tumor diameter larger than 8 cm (p = 0.02) and symptom duration longer than 4 months (p = 0.02) predicted inferior LCR in the group with localized tumor.

CONCLUSIONS

This is a single-institutional experience of 84 patients with thoracic PNETs who underwent a uniform chemotherapy protocol. Novel prognostic factors were identified for thoracic PNET. All efforts should be made to resect primary tumor after neoadjuvant chemotherapy because radical RT results in inferior EFS and LCR despite good response to neoadjuvant chemotherapy.

摘要

背景

关于采用统一化疗方案治疗的胸原始神经外胚层肿瘤（PNET）的数据在文献中很少。我们分析了接受胸 PNET 治疗的患者的结局和预后因素。

方法

这是一项对 2003 年 6 月至 2011 年 11 月期间接受统一新辅助化疗、手术干预或放疗（RT）或这些治疗方法联合作为局部治疗后辅助化疗的胸 PNET 患者的单机构数据回顾。

结果

在 374 例 PNET 患者中发现了 84 例胸 PNET，中位年龄为 15 岁（范围 3-40 岁）；其中 27 例（32%）有转移。30 例患者接受了手术切除；27 例患者在新辅助化疗后接受根治性 RT。根治性 RT 组无不良肿瘤特征或对新辅助化疗反应不佳。中位随访 20.8 个月（范围 2-104.6 个月）时，整个队列的 5 年无事件生存率（EFS）、总生存率（OS）和局部控制率（LCR）分别为 24.4%±5.9%、47.9%±8.4%和 59.3%±9%，局限性肿瘤组分别为 31%±7.7%、59%±10.4%和 67%±9.7%。多变量分析显示，症状持续时间超过 4 个月（p=0.03）、起源于骨骼的原发性肿瘤（p=0.03）和根治性 RT（p=0.006）预测整个队列和局限性疾病患者的 EFS 更差；转移性疾病（p=0.002）预测 OS 更差。根治性 RT 预测整个队列和局限性肿瘤组的 LCR 更差；肿瘤直径大于 8cm（p=0.02）和症状持续时间超过 4 个月（p=0.02）预测局限性肿瘤组的 LCR 更差。