背景:原发性胸壁肿瘤起源于肌肉,脂肪,血管,神经鞘,软骨,或者胸壁的骨头.胸壁肉瘤之一是尤因肉瘤(ES),1921年由詹姆斯·尤因首次描述,这是一种高度侵袭性的骨和软组织癌症。此病例报告旨在介绍年轻成年男性患者的Ewing肉瘤,并伴有胸内和多个胸外转移。
方法:我们描述了一个23岁男性的尤因肉瘤转移的独特病例,该病例显示右后下肺有胸腔积液,最初被认为是转移到胸膜的肺部肿瘤。胸部CT扫描显示右后外侧胸壁有一个分叶状软组织肿块,或者胸膜,肋骨上的软组织肿块扩张。胸部MRI显示右下胸壁后区肿瘤,左侧肋骨转移,右侧胸腔积液伴右肺下叶肺不张。病人还接受了骨骼扫描,计划进行姑息性放疗和化疗,并咨询了肿瘤外科医生。
结论:尤因肉瘤是一个小的,圆形,蓝色细胞间充质恶性肿瘤。ES主要影响儿童,青少年,和年轻人,每百万儿童>1.5例。男性比女性受到的影响略大(性别比为3:2)。明确的诊断需要活检证明(通过细针或核心活检)。最常见的转移区域是肺,胸膜腔,骨骼系统,骨髓,或这些的组合。
结论:当没有转移时,5年生存率约为70%;当存在转移时,该生存率下降到30%左右。
BACKGROUND: Primary chest wall tumors arise from muscle, fat, blood vessels, the nerve sheath, cartilage, or bone of the chest wall. One of the chest wall sarcomas is Ewing Sarcoma (ES), first described in 1921 by James Ewing, which is a highly aggressive bone and soft-tissue cancer. This
case report aimed to present an Ewing Sarcoma with intra thoracic and multiple extra thoracic metastases in young adult male patient.
METHODS: We describe a unique
case of metastatic of ewing\'s sarcoma in a 23-year-old male that showed a mass on the right lower posterior lung with pleural effusion, which was initially thought to be lung tumor that metastasized to the pleura. A thoracic CT scan showed a lobulated soft tissue mass on the right posterolateral thoracic wall, or pleura, with an expansion of soft tissue mass on the rib. Thoracal MRI showed tumor in the posterior right lower thoracic wall area, metastases of the left lateral rib, and right pleural effusion with atelectasis in the right inferior lobe of the lung. The patient also underwent a bone scan, scheduled for palliative radiotherapy and chemotherapy, and consulted to oncology surgeon.
CONCLUSIONS: Ewing sarcoma is a small, round, blue-cell mesenchymal malignancy. ES mainly affects children, adolescents, and young adults, with >1.5 cases per million children. Males are slightly more affected than females (sex ratio of 3:2). The definitive diagnosis requires biopsy proof (achieved by fine needle or core biopsy). The most common regions of metastasis are the lungs, pleural cavity, skeletal system, bone marrow, or combinations of these.
CONCLUSIONS: The 5-year survival rate is approximately 70 % when there is no metastasis; this rate falls to around 30 % when metastasis is present.