Multimodality imaging features, metastatic pattern and clinical outcome in adult extraskeletal Ewing sarcoma: experience in 26 patients
Multimodality imaging features, metastatic pattern and clinical outcome in adult extraskeletal Ewing sarcoma: experience in 26 patients
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DOI：
10.1259/bjr.20140123
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发表时间：
2014-06-01
影响因子：
2.6
通讯作者：
Tirumani, S. H.
中科院分区：
文献类型：
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作者：
Somarouthu, B. S.;Shinagare, A. B.;Tirumani, S. H.
目的：描述成人骨外尤文肉瘤 (EES) 的多模态成像特征、转移模式和临床结果。方法：在这项经机构审查委员会批准、符合健康保险可移植性和责任法案的回顾性研究中，我们纳入了 26 名患者（17 名女性和 9 名男性；平均年龄，36 岁；范围，18-85 岁），这些患者于 1999 年至 1999 年间在我们研究所见过经病理证实的 EES 2011年，对原发肿瘤进行了影像学检查。两名放射科医生一致审查了所有 26 名患者的原发肿瘤影像学和 23 名患者的后续影像学检查。临床数据从电子病历中提取。结果：最常见的原发部位是躯干（n = 13）、四肢（n = 10）和头颈（HN）区域（n = 3）。肿瘤平均大小为 9 cm（范围，3-22 cm）；躯干的肿瘤大于其他部位的肿瘤（p > 0.05）。与骨骼肌相比，肿瘤在 CT 上呈等密度 (21/21)，在 T-1 加权图像上呈低信号 (n = 5) 至等信号 (n = 14)，在 T-2 加权图像上呈高信号 (19/19)，并且富含氟 18 氟脱氧葡萄糖 (F-18-FDG) [10/10；平均最大标准化摄取值为 7（范围，3-11）]。存在坏死（15/26）、出血（5/26）和邻近器官侵犯（14/26），无钙化。中位随访时间为 16 个月。 5 名患者出现局部复发（躯干，3 名；四肢，1 名；HN，1 名）。 11 名患者出现转移（躯干，7 名；四肢，3 名；HN，1 名；p > 0.05）； 8 就诊时，最常见于肺 (9/11)、腹膜 (4/11)、肌肉 (4/11) 和淋巴结 (4/11)。 9 例患者（躯干 7 例；四肢 1 例；HN 1 例）死亡（中位生存期 10 个月）（p > 0.05）。结论：成人 EES 是大肿瘤，经常侵犯邻近器官并转移至肺部。躯干的 EES 较大，转移更频繁，预后较差。知识进展：与其他 EES 相比，成人躯干 EES 的预后较差。
Objective: To describe the multimodality imaging features, metastatic pattern and clinical outcome in adult extraskeletal Ewing sarcoma (EES).Methods: In this institutional reviewboard-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 T-1 weighted image, hyperintense on T-2 weighted image (19/19) and were fluorine-18 fludeoxyglucose (F-18-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.