Clinical analysis of 21 cases of primary Ewing sarcoma of the thoracic wall

Lili JIANG; Yan MA; Tiantian ZHANG; Shan HUANG

doi:10.19401/j.cnki.1007-3639.2024.01.004

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Clinical analysis of 21 cases of primary Ewing sarcoma of the thoracic wall

Article | 更新时间：2025-12-31

- Clinical analysis of 21 cases of primary Ewing sarcoma of the thoracic wall
- China Oncology Vol. 34, Issue 1, Pages: 74-81(2024)
- 作者机构：
  
  苏州大学附属第一医院病理科，江苏苏州 215000
- 作者简介：
  
  HUANG Shan.
- 基金信息：
- DOI：10.19401/j.cnki.1007-3639.2024.01.004
  CLC： R738.6
- Received：02 August 2023，
  
  Revised：2023-12-20，
  
  Published：30 January 2024
- 稿件说明：
移动端阅览
Lili JIANG, Yan MA, Tiantian ZHANG, et al. Clinical analysis of 21 cases of primary Ewing sarcoma of the thoracic wall[J]. China Oncology, 2024, 34(1): 74-81.
DOI：

Lili JIANG, Yan MA, Tiantian ZHANG, et al. Clinical analysis of 21 cases of primary Ewing sarcoma of the thoracic wall[J]. China Oncology, 2024, 34(1): 74-81. DOI： 10.19401/j.cnki.1007-3639.2024.01.004.

摘要

背景与目的：

原发性胸壁尤因肉瘤（primary Ewing sarcoma of the thoracic wall，PEST）是发生在胸壁或胸腔的罕见骨外尤因肉瘤。PEST患者生存期短，预后差，复发率高。PEST发病原因未明，发展迅速，早诊断、早治疗是提高患者生存率的有效手段。本研究通过收集PEST病例，探讨其临床病理学特征、治疗方法及预后，以提高临床对该病的认识及诊疗水平。

方法：

回顾性分析2018—2023年苏州大学附属第一医院收治的及文献报道的共21例PEST患者的临床资料、病理学特征、治疗及随访结果，采用Kaplan-Meier法计算年龄与累积生存率的关系。

结果：

全组PEST患者男女比例为13:8，左右胸比例为6:15，中位年龄为20岁，平均年龄为28岁，中位肿瘤直径为8.0 cm，平均肿瘤直径为18.1 cm。65.2%患者同侧胸腹痛，47.6%患者侵犯同侧肋骨伴胸腔积液。病理学检查符合肿瘤特征，瘤细胞呈紧密成片或小叶状分布的蓝色小圆细胞，100% CD99弥漫强阳性，80%波形蛋白（vimentin）阳性。苏州大学附属第一医院收治的2例患者荧光原位杂交（fluorescence

in situ

hybridizati

on，FISH）检测可见EWSR1分离信号，二代测序（next-generation sequencing，NGS）可见

EWSR

FLI

1融合。2例患者术前接受新辅助化疗，10例术后接受放化疗，5例仅接受放化疗，1例仅接受手术治疗，3例无手术资料。全组患者随访时间为3~38个月，7例失访。患者的发病年龄与累积生存率呈正相关，平均生存时间为19.98个月，中位生存时间为13.00个月。

结论：

PEST多见于年轻男性，易发生于右胸，肿物常大于8 cm，大部分病例可通过病理形态学、免疫表型初诊，FISH或NGS行

EWSR

1基因检测可提供精确诊断。PEST患者预后极差，累积生存率与发病年龄呈正相关，手术、放疗及化疗为PEST的主要治疗手段。

Abstract

Background and Purpose:

Primary Ewing sarcoma of the thoracic wall (PEST) is a rare extraosseous Ewing sarcoma that occurs in the chest wall or thoracic cavity with a short survival

poor prognosis and a high rate of recurrence. Early diagnosis and treatment are the best way to prolong survival time since the cause of PEST is not clear. This study aimed to explore the clinicopathologic characteristics

diagnosis and treatment of PEST to improve clinical understanding of this disease.

Methods:

A total of 21 cases with PEST were treated at The First Affiliated Hospital of Soochow University

and reviews were published from 2018 to 2023. Clinical data

pathological features

treatment and follow-up of the patients were analyzed respectively. The survival was from the start of treatment to the death of the patient or the end of the follow-up. Cumulative survival was estimated by Kaplan-Meier method.

Results:

A total of 21 cases with PEST (male/female ratio

13:8; sites of left/right chest ratio

6:15; median age

20 years; mean age

28 years; median diameter of the tumor

8.0 cm; mean diameter of the tumor

18.1 cm) met the inclusion criteria. 65.2% of the patients presented with the pain in the ipsilateral thoracic and abdominal area. In 47.1% of cases

the ipsilateral ribs were invaded with pleural effusion. Pathological morphology microscopy showed most tumor cells were tightly packed or lobular distribution of small blue round cells. In immunohistochemistry

CD99 and vimentin were positive in 100% and 80% cases

respectively while neurogenic markers were expressed to varying degrees. EWSR1 separated signal was found by fluorescence

in situ

hybridization (FISH)

and the

EWSR

FLI

1 fusion was detected by next-generation sequencing (NGS) in two cases at our hospital. Two cases received neoadjuvant chemotherapy

10 patients received chemotherapy and radiotherapy after operation

5 cases were treated with radiotherapy only

1 case received surgery only

and 3 cases had no surgical data. A total of 14 cases were followed up for 3-38 month while 7 cases were lost to visit. Cumulative survival correlates with age at disease. The mean survival time was 19.98 months

and the median survival time was 13.00 months.

Conclusion:

Young males

right chest and the mass larger than 8 cm are more often found. Most cases can be initially diagnosed using histopathology and immunohistochemical markers. FISH or NGS of the

EWSR

1 gene test are a highly accurate method for diagnosis. The prognosis of PEST is extremely poor

and the cumulative survival rate is negatively correlated with the age of onset. Surgery

radiotherapy and chemotherapy are the main treatments for this disease.

关键词

Keywords

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