Perilongo G, Massimino M, Sotti G, Belfontali T, Masiero L, Rigobello L, Garrè L, Carli M, Lombardi F, Solero C, Sainati L, Canale V, del Prever A B, Giangaspero F, Andreussi L, Mazza C, Madon E
Pediatric Oncology Division-Pediatric Department, University of Padova, Italy.
Med Pediatr Oncol. 1997 Aug;29(2):79-85. doi: 10.1002/(sici)1096-911x(199708)29:2<79::aid-mpo3>3.0.co;2-o.
The principal aim of this report is to present the results of multivariate analyses conducted to identify clinical prognostic factors in 92 children aged < 16 years with ependymoma (EPD) retrospectively collected in seven Italian centres. They were treated over a 16-year period (1977-1993). Treatment modalities varied. Surgery and radiotherapy (RT) was the "gold standard" management method for the majority of these children. Only in the late 1980s did some of them receive chemotherapy (CT), mainly with vincristine, lomustine (CCNU) and prednisone. The median follow-up of the entire study population is 36 months (average 43 months; range 12 to 214 months). The 10-year overall (OS) and the progression-free survival (PFS) of the study population were 55.5% (CI 41.4-69.4%) and 34.7% (CI 21.4-47.8%), respectively. Age (< 5 years; > 5 years), sex, site (infratentorial vs. supratentorial), histology (anaplastic/malignant vs. non-anaplastic/non-malignant), type of resection (complete vs. incomplete); use and fields of RT, and of CT employed were entered in a multivariate regression model to test their impact on OS and PFS. On univariate analysis, radical surgery, the use of RT and age more than 5 years at the time of diagnosis achieved statistically significant values for predicting long-term OS and PFS. Histology reached marginal statistical significance but only for PFS. When those variables were entered in a multivariate analysis only radical resection (P = 0.00142 and 0.0001) resulted a significant factor for predicting long-term OS and PFS, while the use of RT reached a marginal statistical significance, but only for PFS (P = 0.05). Children who had the tumour completely resected did significantly better than all the others who had less than a complete resection, with a 10-year OS and PFS for the two groups of patients of 69.8% (CI 53-86.5%) and 57.2% (CI 40.3-75%) and of 32.5% (CI 8.5-57.6%) and 11.1% (0-24.4%), respectively. These findings suggest that, for childhood EPD, radical resection should be pursued as much as reasonably possible. Thus, it seems justified proposing for future trials, patient stratification by entity of surgical resection.
本报告的主要目的是呈现对92名年龄小于16岁的室管膜瘤（EPD）患儿进行多因素分析的结果，这些患儿是从意大利七个中心回顾性收集的。他们在16年期间（1977 - 1993年）接受了治疗。治疗方式各不相同。手术和放疗（RT）是这些患儿大多数的“金标准”治疗方法。直到20世纪80年代后期，其中一些患儿才接受化疗（CT），主要使用长春新碱、洛莫司汀（CCNU）和泼尼松。整个研究人群的中位随访时间为36个月（平均43个月；范围12至214个月）。该研究人群的10年总生存率（OS）和无进展生存率（PFS）分别为55.5%（CI 41.4 - 69.4%）和34.7%（CI 21.4 - 47.8%）。年龄（<5岁；>5岁）、性别、部位（幕下与幕上）、组织学（间变性/恶性与非间变性/非恶性）、切除类型（完全切除与不完全切除）；RT的使用及照射野，以及所采用的CT均纳入多因素回归模型，以测试它们对OS和PFS的影响。单因素分析显示，根治性手术、RT的使用以及诊断时年龄大于5岁对预测长期OS和PFS具有统计学显著意义。组织学仅对PFS达到边缘统计学意义。当将这些变量纳入多因素分析时，仅根治性切除（P = 0.00142和0.0001）是预测长期OS和PFS的显著因素，而RT的使用仅对PFS达到边缘统计学意义（P = 0.05））。肿瘤完全切除的患儿明显比所有其他切除不完全的患儿情况要好，两组患者的10年OS和PFS分别为69.8%（CI 53 - 86.5%）和57.2%（CI 40.3 - 75%）以及32.5%（CI 8.5 - 57.6%）和11.1%（0 - 24.4%）。这些发现表明，对于儿童EPD，应尽可能合理地进行根治性切除。因此，似乎有理由在未来的试验中根据手术切除程度对患者进行分层。
