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成人尤因肉瘤家族性肿瘤的临床结局及预后因素:单中心经验

Clinical outcomes and prognostic factors of adult's Ewing sarcoma family of tumors: single center experience.

作者信息

Uyeturk Ummugul, Helvaci Kaan, Demirci Ayse, Sonmez Ozlem Uysal, Turker Ibrahim, Afsar Cigdem Usul, Budakoglu Burcin, Arslan Ulku Yalcintas, Oksuzoglu Omur Berna, Zengin Nurullah

机构信息

Department of Medical Oncology, Abant Izzet Baysal University, Faculty of Medicine, Bolu, Turkey.

Department of Medical Oncology, Ankara Oncology Education and Research Hospital, Ankara, Turkey.

出版信息

Contemp Oncol (Pozn). 2016;20(2):141-6. doi: 10.5114/wo.2016.58487. Epub 2016 Mar 21.

Abstract

AIM OF THE STUDY

Aim of the study was to investigate the demographics of Ewing sarcoma family of tumours (ESTF) patients, treatment alternatives, clinical outcomes, and prognostic factors for survival.

MATERIAL AND METHODS

We retrospectively reviewed 39 patients with ESFT who were admitted to our institute between September 2008 and September 2012.

RESULTS

The patients included 32 (82.1%) males and seven (17.9%) females of median age 24 (range, 18-66) years. Among the 27 patients with a primary osseous localization, 17 (43.5%) had a central axis localization. Fifteen patients (38.5%) had metastases at the time of diagnosis. Patients were followed up for a median period of 18 (range, 2-134) months. The median event-free survival (EFS) was 23 (range, 1-64) months, and the 1- and 4-year EFS were 60% and 48%, respectively. The median overall survival (OS) was 91 (range, 1-188) months, and the 1- and 4-year OS were 78% and 54%, respectively. Gender, age, primary tumor site, and local treatment modalities, either alone or in combination, did not have a significant effect on OS (p = 0.210, p = 0.617, p = 0.644, and p = 0.417, respectively). In contrast, osseous site of peripheral localization, limited stage, and metastasis to the bone significantly affected OS (p = 0.015, p < 0.001, and p = 0.042, respectively).

CONCLUSIONS

ESFTs are aggressive tumors with a high rate of relapse and metastatic potential. Patients with peripheral bone involvement and limited stage had a good prognosis. Appropriate surgical resection, radiotherapy, and aggressive chemotherapy regimens are recommended.

摘要

研究目的

本研究旨在调查尤因肉瘤肿瘤家族(ESTF)患者的人口统计学特征、治疗选择、临床结果以及生存预后因素。

材料与方法

我们回顾性分析了2008年9月至2012年9月间入住我院的39例尤因肉瘤肿瘤家族(ESFT)患者。

结果

患者包括32例(82.1%)男性和7例(17.9%)女性,中位年龄为24岁(范围18 - 66岁)。在27例原发性骨定位患者中,17例(43.5%)为中心轴定位。15例患者(38.5%)在诊断时已有转移。患者中位随访时间为18个月(范围2 - 134个月)。中位无事件生存期(EFS)为23个月(范围1 - 64个月),1年和4年EFS分别为60%和48%。中位总生存期(OS)为91个月(范围1 - 188个月),1年和4年OS分别为78%和54%。性别、年龄、原发肿瘤部位以及局部治疗方式,单独或联合起来,对总生存期均无显著影响(p值分别为0.210、0.617、0.644和0.417)。相比之下,外周定位的骨部位、局限期以及骨转移对总生存期有显著影响(p值分别为0.015、<0.001和0.042)。

结论

尤因肉瘤肿瘤家族(ESFT)是具有高复发率和转移潜能的侵袭性肿瘤。外周骨受累且处于局限期的患者预后良好。建议采用适当的手术切除、放疗和积极的化疗方案。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb22/4925728/56151bd56d15/WO-20-27102-g001.jpg

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