Pizer Barry L, Weston Claire L, Robinson Kathryn J, Ellison David W, Ironside James, Saran Frank, Lashford Linda S, Tait Diana, Lucraft Helen, Walker David A, Bailey Clifford C, Taylor Roger E
Alder Hey's Children's Hospital-Oncology Unit, Eaton Road, Liverpool L12 2AP, UK.
Eur J Cancer. 2006 May;42(8):1120-8. doi: 10.1016/j.ejca.2006.01.039. Epub 2006 Apr 24.
The SIOP PNET 3 study was designed to determine whether 10 weeks of moderately intensive chemotherapy given after surgery and before radiotherapy (RT) would improve the outcome for patients with primitive neuroectodermal tumours (PNETs) compared with RT alone. Patients with a histological diagnosis of supratentorial PNET (StPNET) and no radiological evidence of metastatic disease were initially eligible for randomisation to either chemotherapy followed by craniospinal RT 35 Gy in 21 fractions with a boost of 20 Gy in 12 fractions to the primary site, or RT alone. In respect of the increasing recognition that StPNET were high-risk tumours, randomisation for this group closed in November 1999. This analysis includes both randomised and non-randomised patients with StPNET entered into the study database. Sixty-eight patients aged 2.9-16.6 years (median 6.5 years) were included in the analysis (chemotherapy+RT: 44, RT alone: 24). Fifty-four patients (79%) had a non-pineal and 14 (21%) a pineal site. At a median follow-up of 7.4 years, for all patients overall survival (OS) at 3 and 5 years was 54.4% and 48.3%, respectively. Event-free survival (EFS) at 3 and 5 years was 50.0% and 47.0%, respectively. There was no statistically significant difference in OS or EFS according to treatment received. OS (P=0.05) and EFS (P=0.03) were significantly better for patients with pineal primary sites. EFS for pineal tumours were 92.9% at 3 years and 71.4% at 5 years and for non-pineal primaries 40.7% at 3 years and 40.7% at 5 years. This study confirmed the relatively good survival for non-metastatic pineal PNETs but poor survival of non-pineal StPNETs. There was no evidence that pre-radiation chemotherapy improved outlook. Future treatment programs should be directed at the particular natural history of these tumours, to further define prognostic factors and to explore further biological characteristics.
儿童国际肿瘤学会（SIOP）的PNET 3研究旨在确定，与单纯放疗相比，在手术之后、放疗（RT）之前给予10周的中度强化化疗，是否会改善原始神经外胚层肿瘤（PNET）患者的预后。组织学诊断为幕上PNET（StPNET）且无影像学转移证据的患者，最初有资格随机分组，接受化疗后行全脑全脊髓放疗35 Gy，分21次进行，对原发部位追加放疗20 Gy，分12次进行，或者单纯接受放疗。鉴于越来越多的人认识到StPNET是高危肿瘤，该组的随机分组于1999年11月结束。本分析纳入了进入研究数据库的随机分组和未随机分组的StPNET患者。分析纳入了68例年龄在2.9至16.6岁（中位年龄6.5岁）的患者（化疗+放疗组：44例，单纯放疗组：24例）。54例患者（79%）肿瘤位于非松果体部位，14例（21%）位于松果体部位。中位随访7.4年时，所有患者的3年和5年总生存率（OS）分别为54.4%和48.3%。3年和5年无事件生存率（EFS）分别为50.0%和47.0%。根据接受的治疗，OS和EFS没有统计学上的显著差异。松果体原发部位的患者OS（P=0.05）和EFS（P=0.03）显著更好。松果体肿瘤的EFS在3年时为第92.9%，5年时为71.4%；非松果体原发肿瘤在3年和5年时均为40.7%。本研究证实了非转移性松果体PNET的生存率相对较好，而非松果体StPNET的生存率较差。没有证据表明放疗前化疗能改善预后。未来的治疗方案应针对这些肿瘤的特殊自然史，以进一步明确预后因素并探索更多生物学特征。
