目的：探讨尤文瘤与骨外周性原始神经外胚层瘤（pPNETs）的临床和影像学表现的异同点，为两者的鉴别和治疗提供帮助。方法：回顾性分析18例尤文瘤和10例骨pPNETs的临床和X、CT及MR资料。结果：尤文瘤的年龄范围10~28岁，平均18.5岁。多以局部疼痛（17?蛐18）伴肿胀（13?蛐18）为主诉，1例以局部肿块为主诉。病变的部位为股骨6例、肱骨4例、锁骨2例，胫骨、腓骨、尺骨、髂骨、骶骨及髋臼各1例。18例X线片中，溶骨性骨质破坏7例，溶骨性骨质破坏并不规则骨质增生、密度增高11例；10例见软组织肿块；10出现骨膜反应，其中葱皮样骨膜反应3例；病变呈偏侧性6例；一侧骨皮质出现压迹3例。12例CT扫描中，边界不清的溶骨性骨质破坏4例，溶骨性骨质破坏并不规则骨质增生、密度增高8例；10中有大小不一的软组织肿块形成；8例出现骨膜反应。9例MRI扫描中，T1WI等信号8例，中等偏高信号1例；T2WI及STIR呈不均匀中高信号；8例中见大小不一的软组织肿块形成，肿块的大小为2cm×3cm～3cm×10cm；5例MR增强扫描中，3例呈显著性强化，2例呈不均匀显著性强化。骨外周性原始神经外胚层瘤（pPNETs）年龄范围8~68岁，平均24.4岁。病变的部位为：髂骨3例、肱骨干骺端2例，椎体、椎弓、肩胛骨、骶骨、蝶骨大翼各1例；多以局部疼痛（9?蛐10）伴肿块为（7?蛐10）主诉。X线溶骨性骨质破坏8例，伴有不规则硬化和轻度膨胀的溶骨性骨质破坏1例，未见异常1例；7例见软组织肿块；均未见骨膜反应。7例CT扫描中边界不清的溶骨性骨质破坏6例，伴有不规则硬化和轻度膨胀的溶骨性骨质破坏1例，均有明显的软组织肿块形成，2例伴有细小的点状钙化，均未见骨膜反应；10例MRI扫描中，T1WI等信号9例，中等偏高信号1例；T2WI及STIR不均匀中高信号8例，均匀高信号2例；均见软组织肿块形成，肿块的大小为2.5cm×4.0cm~7.5cm×13.0cm；5例MR增强扫描中，2例呈中等度强化，3例呈不均匀显著性强化。结论：和骨外周性原始神经外胚层瘤比较，尤文瘤的发病年龄相对较轻，以长管骨好发，X线和CT上骨膜反应明显，骨质密度增高多见，MR上其信号改变相似但软组织肿块较小，结合病理检查有助于两者的诊断和鉴别诊断。
Abstract
Objective: To describe the difference of the clinical symptoms and radiologic features between Ewing sarcoma(ES) and peripheral primitive neuroectodermal tumors(pPNETs) of bone, and to benefit from the differential diagnosis and treatment. Materials and Methods: We retrospectively reviewed 18 cases of ES and 10 cases of pPNETs all were confirmed pathologically. Radiologic and clinical studies were reviewed by two musculoskeletal radiologists. In ES, radiographs(n=18), CT(n=12), MRI(n=9) were performed. In pPNETs, radiographs(n=10), CT(n=7), MRI(n=10) were performed too. The evaluation included clinical presentation,lesion location and size,bone erosion/destruction,periosteal reaction, soft tissue involvement and intrinsic characteristic findings on CT and MRI. Results: Patients’ average age was 18.5 years (range 10～28) and 24.4 years(range 8～86) in ES and pPNETs respectively. For the ES, the lesion locations included femur(n=6), humerus(n=4), clavicle(n=2), tibia(n=1), fibula(n=1), ulna(n=1), ilium(n=1), sacrum(n=1) and acetabulum(n=1); Radiographs(n=18) showed lytic lesion(7/18), with irregular sclerosis and matrix mineralization(11/18), soft tissue mass(10/18), periosteal reaction(10/18), eccentric(6/18), scalloping of cortex(3/18); CT(n=12) showed lytic lesion(4/12), lesion with irregular sclerosis and matrix mineralization(8/12), soft tissue mass(10/12), periosteal reaction(6/12); On MRI(n=9), T1WI showed isointensity(8/9) and slightly hyperintensity(1/9), T2WI and STIR images showed heterogeneous hyperintensity(9/9), soft tissue mass(8/9), the size ranged from 2cm×3cm to 3cm×10cm. For the pPNETs, the lesion locations included ilium(n=3), humerus(n=2), corpus vertebrae(n=1), arcus vertebrae(n=1), scapula(n=1), sacrum(n=1), sphenoid(n=1); Radiographs(n=10) showed lytic lesion(8/10), with irregular sclerosis and dilation(1/10), and normal(1/10), soft tissue mass(7/10), periosteal reaction(0/10). CT(n=7) showed lytic lesion(6/7), lytic lesion with irregular sclerosis and dilation (1/7), soft mass(7/7), speculate calcification(2/7), periosteal reaction(0/10); On MRI(n=10), T1WI showed isointensity(9/10) and slightly hyperintensity(1/10), T2WI and STIR images showed heterogeneous hyperintensity (8/10), uniform hyperintensity(2/10), soft tissue mass(10/10), the size ranged from 2.5cm×4cm to 7.5cm×13.5cm. Conclusion: The clinical and radiologic features of ES and pPNETs were different, compared with pPNETs, the patients with ES were younger, the commoner locations were long tubular bones, radiography and CT showed periosteal reaction and matrix mineralization, MRI showed the similar intensity but with smaller soft tissue mass, these features were helpful in differential diagnosis if combining with pathology.
关键词
肉瘤 /
Ewing /
神经外胚瘤 /
原始 /
外周 /
体层摄影术 /
X线计算机 /
磁共振成像
Key words
sarcoma /
Ewing’s /
neuroectodermal tumors /
primitive /
peripheral /
tomography /
X-ray computed /
magnetic resonance imaging
中图分类号：
Ｒ738.1
Ｒ814.42
Ｒ445.2
