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肩胛部尤文肉瘤患者的特征和肿瘤学结局。

Characteristics and oncologic outcomes of patients with Ewing sarcoma of the scapula.

机构信息

Department of Orthopaedics, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, Boston, MA, 02114, USA.

Department of Orthopaedics, La Paz University Hospital, Calle Arturo Soria 270, Madrid, Spain.

出版信息

Surg Oncol. 2021 Sep;38:101619. doi: 10.1016/j.suronc.2021.101619. Epub 2021 Jun 16.

Abstract

BACKGROUND AND OBJECTIVES

Ewing sarcoma is the second most common bone sarcoma of childhood. Ewing sarcomas of the scapula are rare, with little known about their characteristics and outcomes. In this study, we describe the demographic characteristics, tumor characteristics, and oncologic outcomes of patients with Ewing sarcoma of the scapula.

METHODS

This is a retrospective case series of thirty-four patients treated at three urban hospitals between 1993 and 2014 for Ewing sarcomas affecting the scapula. Their demographic data, tumor characteristics, and oncologic outcomes are reported and contrasted with data on Ewing sarcoma described in the literature.

RESULTS

Patients in our case series were 59% male. The average age at diagnosis was 16 years. 44% of patients had metastatic disease at presentation. 26% of patients had a tumor size >8 cm in largest dimension at diagnosis. 9 patients in our series had the t (11; 22) translocation present. Patients had a survival rate of 68% at five years. No patients had local recurrence of disease. Compared with findings reported in the literature concerning Ewing sarcoma affecting other locations, patients with Ewing sarcoma of the scapula were slightly older at time of diagnosis, had a lower percentage of tumors with size > 8 cm in largest dimension at presentation, and more commonly had metastatic disease at presentation. Patients in our cohort had a 5-year survival rate of 68%, which is higher than the rate of approximately 55% as reported in the general literature.

CONCLUSIONS

In this study, we describe a retrospective case series of thirty-four patients with Ewing sarcomas of the scapula. This is the largest case series to date of Ewing sarcoma affecting this location to our knowledge. These results will contribute to the understanding of the clinical profile and oncologic behavior of Ewing sarcomas affecting the scapula.

摘要

背景与目的

尤因肉瘤是儿童期第二常见的骨肉瘤。肩胛骨尤因肉瘤较为罕见,其特征和预后知之甚少。本研究旨在描述肩胛骨尤因肉瘤患者的人口统计学特征、肿瘤特征和肿瘤学结局。

方法

本研究回顾性分析了 1993 年至 2014 年期间在三家市级医院接受治疗的 34 例肩胛骨尤因肉瘤患者的资料。报告了他们的人口统计学数据、肿瘤特征和肿瘤学结局,并与文献中描述的尤因肉瘤数据进行了对比。

结果

本研究中,患者的男女比例为 59%:41%,平均诊断年龄为 16 岁。44%的患者在就诊时即有远处转移,26%的患者就诊时肿瘤最大径>8cm。本研究中有 9 例患者存在 t(11;22)易位。患者的 5 年生存率为 68%。无局部复发病例。与文献中描述的其他部位尤因肉瘤相比,本研究中患者的诊断年龄稍大,就诊时肿瘤最大径>8cm的比例稍低,而就诊时伴有远处转移的比例较高。本研究中患者的 5 年生存率为 68%,高于文献报道的约 55%。

结论

本研究回顾性分析了 34 例肩胛骨尤因肉瘤患者的资料。这是迄今为止我们所知的该部位尤因肉瘤最大的病例系列研究。这些结果有助于了解影响肩胛骨的尤因肉瘤的临床特征和肿瘤学行为。

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