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成人髓母细胞瘤的放射治疗:单机构长期结果。预后因素综述及我们为何需要多机构合作项目。

Radiotherapy for adult medulloblastoma: Long term result from a single institution. A review of prognostic factors and why we do need a multi-institutional cooperative program.

作者信息

Buglione Michela, Ghirardelli Paolo, Triggiani Luca, Pedretti Sara, Pasinetti Nadia, De Bari Berardino, Tonoli Sandro, Borghetti Paolo, Spiazzi Luigi, Magrini Stefano Maria

机构信息

Radiation Oncology Department, Brescia University and Spedali Civili - Brescia, Brescia, Italy.

Medical Physics, Spedali Civili - Brescia, Brescia, Italy.

出版信息

Rep Pract Oncol Radiother. 2015 Jul-Aug;20(4):284-91. doi: 10.1016/j.rpor.2015.03.003. Epub 2015 Apr 8.

Abstract

AIM

We retrospectively analyzed our Institution experience with these patients. The endpoints of the analysis were overall survival (OS), disease-free survival (DFS), local control (LC), metastasis free survival (MFS); results were compared with the literature.

BACKGROUND

Medulloblastoma in adult patients is a very rare disease; the 5 and 10-year overall survival rates range between 33-78% and 27-56%, respectively. The collection of more clinical data is strongly needed.

MATERIALS AND METHODS

From September 1975 to October 2006, we treated 16 adult patients (9 males and 7 females) with a histological diagnosis of medulloblastoma. Acute and late toxicities were scored according to RTOG toxicity scale. Karnofski performance status (KPS) and neurological performance status (NPS) pre- and post-RT were reported. Median age was 27 years (range 18-53 years). All the patients received cranio-spinal irradiation, two patients were also given chemotherapy. Median follow-up period was 121.5 months.

RESULTS

In January 2014, 10/16 patients were alive without evidence of disease, 6/16 died with progressive disease (1 local and spinal, 3 spinal and 2 extraneural). Ten-year LC, OS, DFS, MFS were, respectively, 84%, 67%, 60% and 59%. Univariate analysis shows that gross total resection is associated with better survival. No acute or late G3-G4 toxicity was observed.

CONCLUSIONS

This experience and the analysis of the literature confirm the efficacy of postoperative RT but also the need of large datasets to better define prognostic factors and the possible role of the association of chemotherapy.

摘要

目的

我们回顾性分析了本机构对这些患者的治疗经验。分析的终点指标为总生存期(OS)、无病生存期(DFS)、局部控制率(LC)、无转移生存期(MFS);并将结果与文献进行比较。

背景

成年患者的髓母细胞瘤是一种非常罕见的疾病;5年和10年总生存率分别在33% - 78%和27% - 56%之间。迫切需要收集更多的临床数据。

材料与方法

1975年9月至2006年10月,我们治疗了16例经组织学诊断为髓母细胞瘤的成年患者(9例男性,7例女性)。根据RTOG毒性量表对急性和晚期毒性进行评分。报告放疗前后的卡氏功能状态评分(KPS)和神经功能状态评分(NPS)。中位年龄为27岁(范围18 - 53岁)。所有患者均接受了全脑全脊髓照射,2例患者还接受了化疗。中位随访期为121.5个月。

结果

2014年1月,16例患者中有10例存活且无疾病证据,16例中有6例死于疾病进展(1例局部和脊髓转移,3例脊髓转移,2例神经外转移)。10年局部控制率、总生存率、无病生存率、无转移生存率分别为84%、67%、60%和59%。单因素分析表明,全切除与更好的生存率相关。未观察到急性或晚期3 - 4级毒性反应。

结论

本经验及文献分析证实了术后放疗的疗效,但也表明需要大型数据集来更好地确定预后因素以及化疗联合应用的可能作用。

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