成人骨外尤文肉瘤的多模态影像学特征、转移模式和临床转归：26 例患者的经验。
Multimodality imaging features, metastatic pattern and clinical outcome in adult extraskeletal Ewing sarcoma: experience in 26 patients.
机构信息
1 Department of Imaging, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
出版信息
Br J Radiol. 2014 Jun;87(1038):20140123. doi: 10.1259/bjr.20140123. Epub 2014 Apr 15.
OBJECTIVE
To describe the multimodality imaging features, metastatic pattern and clinical outcome in adult extraskeletal Ewing sarcoma (EES).
METHODS
In this institutional review board-approved, health insurance portability and accountability act-compliant retrospective study, we included 26 patients (17 females and 9 males; mean age, 36 years; range, 18-85 years) with pathologically confirmed EES seen at our institute between 1999 and 2011, who had imaging of primary tumour. Imaging of primary tumour in all 26 patients and follow-up imaging in 23 was reviewed by two radiologists in consensus. Clinical data were extracted from electronic medical records.
RESULTS
The most common primary sites were the torso (n = 13), extremities (n = 10) and head and neck (HN) region (n = 3). The mean tumour size was 9 cm (range, 3-22 cm); tumours of the torso were larger than those of other areas (p > 0.05). Compared with the skeletal muscle, tumours were isodense on CT (21/21), hypointense (n = 5) to isointense (n = 14) on T1 weighted image, hyperintense on T2 weighted image (19/19) and were fluorine-18 fludeoxyglucose ((18)F-FDG)-avid [10/10; mean maximum standardized uptake value of 7 (range, 3-11)]. Necrosis (15/26), haemorrhage (5/26) and adjacent organ invasion (14/26) were present without calcification. Median follow-up was 16 months. 5 patients had local recurrence (torso, 3; extremity, 1; and HN, 1). Metastases developed in 11 patients (torso, 7; extremities, 3; and HN, 1; p > 0.05); 8 at presentation, most commonly to lung (9/11), peritoneum (4/11), muscles (4/11) and lymph nodes (4/11). Nine patients (torso, 7; extremity, 1; and HN, 1) died (10 months median survival) (p > 0.05).
CONCLUSION
Adult EESs are large tumours, which frequently invade adjacent organs and metastasize to the lung. EESs of the torso are larger, have more frequent metastases and poorer outcomes.
ADVANCES IN KNOWLEDGE
Adult EESs of the torso have poor outcomes compared with other EESs.
目的
描述成人骨外尤文肉瘤（EES）的多模态影像学特征、转移模式和临床结局。
方法
在这项经机构审查委员会批准、符合《健康保险流通与责任法案》的回顾性研究中，我们纳入了 1999 年至 2011 年在我院就诊的 26 例经病理证实的 EES 患者（17 名女性，9 名男性；平均年龄 36 岁；范围 18-85 岁），这些患者均接受了原发肿瘤的影像学检查。由两位放射科医生对 26 例患者的原发肿瘤影像学检查和 23 例患者的随访影像学检查进行了共识性回顾。从电子病历中提取临床数据。
结果
最常见的原发部位为躯干（n = 13）、四肢（n = 10）和头颈部（HN）区域（n = 3）。肿瘤平均大小为 9cm（范围 3-22cm）；与其他部位相比，躯干肿瘤更大（p > 0.05）。与骨骼肌相比，CT 上肿瘤等密度（21/21），T1 加权图像上呈低信号（n = 5）至等信号（n = 14），T2 加权图像上呈高信号（19/19），氟-18 氟脱氧葡萄糖（18）F-FDG 摄取[10/10；最大标准化摄取值的平均值为 7（范围 3-11）]。无钙化表现，可见坏死（15/26）、出血（5/26）和邻近器官侵犯（14/26）。中位随访时间为 16 个月。5 例患者出现局部复发（躯干 3 例，四肢 1 例，HN 1 例）。11 例患者发生转移（躯干 7 例，四肢 3 例，HN 1 例；p > 0.05）；在就诊时就出现了 8 例转移，最常见的转移部位为肺（9/11）、腹膜（4/11）、肌肉（4/11）和淋巴结（4/11）。9 例患者死亡（7 例躯干，1 例四肢，1 例 HN；中位生存时间 10 个月）（p > 0.05）。
结论
成人 EES 为大肿瘤，常侵犯邻近器官，并向肺部转移。躯干 EES 更大，更常发生转移，预后更差。
知识的进步
与其他 EES 相比，躯干 EES 患者的预后较差。
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