Imaging Features of Primary Tumors and Metastatic Patterns of the Extraskeletal Ewing Sarcoma Family of Tumors in Adults: A 17-Year Experience at a Single Institution.

Imaging Features of Primary Tumors and Metastatic Patterns of the Extraskeletal Ewing Sarcoma Family of Tumors in Adults: A 17-Year Experience at a Single Institution.
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DOI:
10.3348/kjr.2015.16.4.783
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发表时间:
2015-07
影响因子:
4.8
通讯作者:
Ramaiya NH
Ramaiya NH
中科院分区:
医学2区
文献类型:
--
作者:
Huh J;Kim KW;Park SJ;Kim HJ;Lee JS;Ha HK;Tirumani SH;Ramaiya NH

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综合分析成人骨骼外尤因肉瘤家族肿瘤(EES)的原发肿瘤和转移模式的影像学特征。我们使用尤文氏肉瘤和原始神经外胚层肿瘤的代码以及年龄≥18岁的人口统计学代码,对我院1996年至2013年的数据仓库进行了计算机检索。我们选择了组织学上被证实患有骨骼外起源的尤文氏肉瘤的受试者。评估原发肿瘤和转移性疾病的影像学特征,包括病变位置、大小、增强模式、坏死、边缘和邻近器官的侵犯。我们纳入的70例患者(平均年龄35.8±15.6岁,范围18-67岁)中,EES原发肿瘤发生在软组织和四肢(n = 20)、腹部和骨盆(n = 18)、胸腔(n = 14)、椎旁间隙(n = 8)、头颈部(n = 6),原发部位未知(n = 4)。原发肿瘤多表现为软组织大而粗大的肿块(平均9.0 cm,范围1.3 ~ 23.0 cm),常侵犯邻近器官(45.6%),表现为不均匀强化(73.7%),边界明确(66.7%),部分坏死/囊性变性(81.9%)。值得注意的是,29名患者在最初诊断时发现了转移性疾病。最常见的转移部位为淋巴结(75.9%),其次为骨(31.0%)、肺(20.7%)、腹部实体器官(13.8%)、腹膜(13.8%)、胸膜(6.9%)和脑(3.4%)。原发性EES肿瘤可发生于任何地方,多表现为大而笨重的软组织肿块。淋巴结是最常见的转移部位。
To comprehensively analyze the spectrum of imaging features of the primary tumors and metastatic patterns of the Extraskeletal Ewing sarcoma family of tumors (EES) in adults. We performed a computerized search of our hospital's data-warehouse from 1996 to 2013 using codes for Ewing sarcoma and primitive neuroectodermal tumors as well as the demographic code for ≥ 18 years of age. We selected subjects who were histologically confirmed to have Ewing sarcoma of extraskeletal origin. Imaging features of the primary tumor and metastatic disease were evaluated for lesion location, size, enhancement pattern, necrosis, margin, and invasion of adjacent organs. Among the 70 patients (mean age, 35.8 ± 15.6 years; range, 18-67 years) included in our study, primary tumors of EES occurred in the soft tissue and extremities (n = 20), abdomen and pelvis (n = 18), thorax (n = 14), paravertebral space (n = 8), head and neck (n = 6), and an unknown primary site (n = 4). Most primary tumors manifested as large and bulky soft-tissue masses (mean size, 9.0 cm; range, 1.3-23.0 cm), frequently invading adjacent organs (45.6%) and showed heterogeneous enhancement (73.7%), a well-defined (66.7%) margin, and partial necrosis/cystic degeneration (81.9%). Notably, 29 patients had metastatic disease detected at their initial diagnosis. The most frequent site of metastasis was lymph nodes (75.9%), followed by bone (31.0%), lung (20.7%), abdominal solid organs (13.8%), peritoneum (13.8%), pleura (6.9%), and brain (3.4%). Primary tumors of EES can occur anywhere and mostly manifest as large and bulky, soft-tissue masses. Lymph nodes are the most frequent metastasis sites.
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