Soeroso Noni Novisari, Ramadhani Nurul, Tarigan Setia Putra
Thoracic Oncology Division, Department of Pulmonology & Respiratory Medicine, Faculty of Medicine, Universitas Sumatera Utara, Chairuddin P. Lubis Universitas Sumatera Utara Hospital, Jl. Dr. Mansur No. 5, Medan, 20155, Indonesia.
Department of Pulmonology & Respiratory Medicine, Faculty of Medicine, Universitas Sumatera Utara, Haji Adam Malik General Hospital, Jl. Dr. Mansur No. 5, Medan, 20155, Indonesia.
Int J Surg Case Rep. 2024 May;118:109642. doi: 10.1016/j.ijscr.2024.109642. Epub 2024 Apr 20.
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.
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.
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.
The 5-year survival rate is approximately 70 % when there is no metastasis; this rate falls to around 30 % when metastasis is present.
原发性胸壁肿瘤起源于胸壁的肌肉、脂肪、血管、神经鞘、软骨或骨骼。胸壁肉瘤之一是尤因肉瘤(ES),1921年由詹姆斯·尤因首次描述,它是一种侵袭性很强的骨和软组织癌症。本病例报告旨在介绍一名年轻成年男性患者发生的伴有胸腔内和多处胸外转移的尤因肉瘤。
我们描述了一名23岁男性尤因肉瘤转移的独特病例,该患者右下肺后部有一个伴有胸腔积液的肿块,最初被认为是转移至胸膜的肺部肿瘤。胸部CT扫描显示右后外侧胸壁或胸膜上有一个分叶状软组织肿块,肋骨上的软组织肿块有增大。胸部MRI显示右后下胸壁区域有肿瘤,左侧肋骨有转移,右侧胸腔积液伴右下肺肺不张。患者还接受了骨扫描,计划进行姑息性放疗和化疗,并咨询了肿瘤外科医生。
尤因肉瘤是一种小型圆形蓝细胞间叶性恶性肿瘤。ES主要影响儿童、青少年和年轻成年人,每百万儿童中发病超过1.5例。男性受影响略多于女性(性别比为3:2)。明确诊断需要活检证据(通过细针或粗针活检获得)。最常见的转移部位是肺、胸腔、骨骼系统、骨髓或这些部位的组合。
无转移时5年生存率约为70%;有转移时该比率降至约30%。