肾脏原发原始神经外胚层肿瘤的影像学表现及病理分析
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摘要
目的:探讨肾脏原发原始神经外胚层肿瘤的病理及影像学特征,以提高诊断水平。方法:回顾性分析6例经手术病理证实的肾脏PNET的影像及临床资料。结果:6例肾脏PNET中,4例位于左肾,2例位于右肾;1例呈类圆形;2例呈椭圆形;1例呈葫芦形;2例呈不规则形;瘤体直径7.0~20.9cm。CT或MRI扫描6例均呈囊实性肿块,CT或MRI平扫6例呈混杂低密度或混杂高低不等信号。增强扫描皮髓质期6例均呈轻度强化,强化方式呈分隔状、条片状、絮状或不均质强化,实质期持续性强化或轻度延迟性强化;2例病灶边界较模糊,其余4例边界清晰。结论:肾脏原发PNET临床罕见,CT和MRI表现对本病的诊断具有一定的提示作用,但确诊仍需要依靠病理学检查。
Objective:To improve diagnostic level by investigating the radiological features and corresponding pathological changes of primary primitive neuroectodermal tumor(PNET)of kidney.Methods:Imaging features and clinical data of Six patients with pathologically proved renal PNET were retrospectively analyzed.Results:Among 6 cases,4 cases were located in the left kidney and 2 cases in the right kidney.One case showed round pattern,2 cases showed oval form,one case showed gourd-shape,and the last 2 were irregular masses.The tumors were 7.0~20.9 cm in diameters.All cases presented as cystic and solid mass in CT or MRI.The solid parts of the 6 cases all showed heterogeneous hypodensity on CT or heterogeneous signal on MRI.The enhancement patterns of the 6 cases were various,including septated,sliced,floccose,and heterogenous.The solid parts of 6 cases showed mild enhancement in corticomedullary phase without exception and they showed mild continuous enhancement in nephrographic phase.There were 2 tumors with ill-defined margin,the other 4 cases were well-defined.Conclusion:Primary PNET of the kidney is rare,CT and MRI can show valuable characteristics of the tumor and provide some help for the diagnosis,but the definitve diagnosis depends on the pathological examination.
Objective:To improve diagnostic level by investigating the radiological features and corresponding pathological changes of primary primitive neuroectodermal tumor(PNET)of kidney.Methods:Imaging features and clinical data of Six patients with pathologically proved renal PNET were retrospectively analyzed.Results:Among 6 cases,4 cases were located in the left kidney and 2 cases in the right kidney.One case showed round pattern,2 cases showed oval form,one case showed gourd-shape,and the last 2 were irregular masses.The tumors were 7.0~20.9 cm in diameters.All cases presented as cystic and solid mass in CT or MRI.The solid parts of the 6 cases all showed heterogeneous hypodensity on CT or heterogeneous signal on MRI.The enhancement patterns of the 6 cases were various,including septated,sliced,floccose,and heterogenous.The solid parts of 6 cases showed mild enhancement in corticomedullary phase without exception and they showed mild continuous enhancement in nephrographic phase.There were 2 tumors with ill-defined margin,the other 4 cases were well-defined.Conclusion:Primary PNET of the kidney is rare,CT and MRI can show valuable characteristics of the tumor and provide some help for the diagnosis,but the definitve diagnosis depends on the pathological examination.
引文
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[12]Dong J,Xing J,Limbu HH,et al.CT Features and pathological correlation of primitive neuroectodermal tumor of the kidney[J].Cell Biochem Biophys,2015,73(1):59-64.
[13]Yang C,Xu H,Zhou J,et al.Renal primitive neuroectodermal tumor:a case report[J].Medicine(Baltimore),2015,94(49):e2304.
[14]Ibarburen C,Haberman JJ,Zerhouni EA.Peripheral primitive neuroectodermal tumors.CT and MRI evaluation[J].Eur J Radiol,1996,21(3):225-232.
[2]梁晓超,赵振华,王伯胤.肾脏Ewing肉瘤或原始神经外胚层瘤的CT表现及与病理对照[J].中华放射学杂志,2014,48(1):61-63.
[3]Ferrozzi F,Tognini G,Marchesi G,et al.Primitive neuroectodermal tumor of the kidney.Magnetic resonance features of a case[J].Radiol Med,2000,99(6):489-491.
[4]Lam JS,Hensle TW,Debelenko L,et al.Organ-confined primitive neuroectodermal tumor arising from the kidney[J].J Pediatr Surg,2003,38(4):619-621.
[5]Li X,Zhang W,Song T,et al.Primitive neuroectodermal tumor arising in the abdominopelvic region:CT features and pathology characteristics[J].Abdom Imaging,2011,36(5):590-595.
[6]Doerfler O,Reittner P,Groell R,et al.Peripheral primitive neuroectodermal tumour of the kidney:CT findings[J].Pediatr Radiol,2001,31(2):117-119.
[7]Akkaya Z,Peker E,Gulpinar B,et al.CT and MRI findings in a rare case of renal primitive neuroectodermal tumor[J].Pol J Radiol,2016,81(4):401-406.
[8]Zokalj I,Igrec J,Plesnar A.Primary renal primitive neuroectodermal tumor/Ewing's sarcoma imaging and pathologic findings of a patient with a nine year,eight month disease free period:case report and review of literature[J].Nephrourol Mon,2016,8(4):e37950.
[9]Kim MS,Kim B,Park CS,et al.Radiologic findings of peripheral primitive neuroectodermal tumor arising in the retroperitoneum[J].AJR,2006,186(4):1125-1132.
[10]Thomas JC,Sebek BA,Krishnamurthi V.Primitive neuroectodermal tumor of the kidney with inferior vena cava and atrial tumor thrombus[J].J Urol,2002,168(4Pt 1):1486-1487.
[11]刘国顺,李雯曦,谌丹丹,等.外周性原始神经外胚层肿瘤的CT及MRI诊断[J].放射学实践,2016,31(10):928-933.
[12]Dong J,Xing J,Limbu HH,et al.CT Features and pathological correlation of primitive neuroectodermal tumor of the kidney[J].Cell Biochem Biophys,2015,73(1):59-64.
[13]Yang C,Xu H,Zhou J,et al.Renal primitive neuroectodermal tumor:a case report[J].Medicine(Baltimore),2015,94(49):e2304.
[14]Ibarburen C,Haberman JJ,Zerhouni EA.Peripheral primitive neuroectodermal tumors.CT and MRI evaluation[J].Eur J Radiol,1996,21(3):225-232.