肝上皮样血管内皮瘤影像学表现及征象分析
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摘要
目的探讨肝上皮样血管内皮瘤(EHE)的影像学表现,提高对该少见病的CT、MRI征象认识,帮助治疗前诊断。方法病理证实肝上皮样血管瘤5例,发病年龄27~55岁;女性3例、男性2例。3例行平扫加螺旋CT动态增强扫描,1例同时行MRI平扫加动态扫描,1例仅行MRI动态扫描。2例间隙性右上腹痛;3例无症状,为体检发现。3例同时伴发肝外病变(多发肺结节2例、腹膜结节1例)。影像学表现与临床病理资料进行对照分析。结果肝周围及包膜下弥漫性病变5例(5/5),其中多发结节3例、多发结节伴大块融合灶2例;伴肝包膜回缩征5例(5/5),肝内静脉进入并止于病灶5例(5/5),病灶包绕血管2例(2/5)。CT平扫表现为多发低密度结节,内见更低密度区,边界清楚(5/5);动态增强典型病灶中层环形强化伴周围低密度晕环,中心不强化(多层靶征)(5/5),延迟期无廓清(5/5),延迟10 min病灶缩小(1/1)。MRI平扫T1WI低信号灶,内见更低信号灶;T2WI高信号灶,内见更高信号;造影后可见多层靶征(2/2)。结论本组肝EHE具有相似的影像学特征,易误诊为肝转移瘤或血管瘤。对首次就诊无肝外恶性肿瘤病史的患者,CT或MRI发现肝周围或包膜下多发肿块或大块融合病灶伴肝包膜回缩者,鉴别诊断时要考虑到血管内皮瘤;造影后持续靶样强化伴延迟强化,合并肝内静脉进入并止于病灶者可提示该病。
Objective To investigate the imaging appearances of hepatic epithelioid hemangioendothelioma(EHE), and to improve the differential diagnosis of hepatic EHE by CT and MRI. Methods Five pathologically confirmed cases of hepatic EHE were enrolled. 3 cases underwent CT scans, 1 underwent combination of CT scans and MRI scans, and 1 underwent MRI scans. The imaging appearances and clinical data were retrospectively analyzed. Results All 5 cases had diffuse and multifocal nodules in the liver periphery and capsule, and 2 cases were accompanied by large confluent masses with a tendency to coalesce. All 5 cases showed the sign of hepatic capsular retraction with lesions encroaching upon veins. Dynamic-enhanced CT scans showed the ring-like enhancement surrounded by hypodense halo in the medium layer. The central area was not enhanced(multilayer target sign). There was no clearance in the delayed phase. The persistent enhancement and multilayer target sign were also shown on contrast-enhanced MRI. Conclusion The imaging features of hepatic EHE lesions are similar to those of hepatic metastatic lesions or hemangiomas. Many published cases are initially misdiagnosed. The following signs may suggest diagnosis of hepatic EHE: peripheral or capsular multiple masses, or coalescing masses with capsular retraction on CT or MRI; persistent target-like enhancement accompanied by delayed enhancement; and lesions encroaching upon veins.
Objective To investigate the imaging appearances of hepatic epithelioid hemangioendothelioma(EHE), and to improve the differential diagnosis of hepatic EHE by CT and MRI. Methods Five pathologically confirmed cases of hepatic EHE were enrolled. 3 cases underwent CT scans, 1 underwent combination of CT scans and MRI scans, and 1 underwent MRI scans. The imaging appearances and clinical data were retrospectively analyzed. Results All 5 cases had diffuse and multifocal nodules in the liver periphery and capsule, and 2 cases were accompanied by large confluent masses with a tendency to coalesce. All 5 cases showed the sign of hepatic capsular retraction with lesions encroaching upon veins. Dynamic-enhanced CT scans showed the ring-like enhancement surrounded by hypodense halo in the medium layer. The central area was not enhanced(multilayer target sign). There was no clearance in the delayed phase. The persistent enhancement and multilayer target sign were also shown on contrast-enhanced MRI. Conclusion The imaging features of hepatic EHE lesions are similar to those of hepatic metastatic lesions or hemangiomas. Many published cases are initially misdiagnosed. The following signs may suggest diagnosis of hepatic EHE: peripheral or capsular multiple masses, or coalescing masses with capsular retraction on CT or MRI; persistent target-like enhancement accompanied by delayed enhancement; and lesions encroaching upon veins.
引文
[1]FURUI S,ITAI Y,OHTOMO K,et al.Hepatic epithelioid hemangioendothelioma:report of five cases[J].Radiology,1989,171(1):63-68.
[2]LYBURN I D,TORREGGIANI W C,HARRIS A C,et al.Hepatic epithelioid hemangioendothelioma:sonographic,CT,and MR imaging appearances[J].Am J Roentgenol,2003,180(5):1359-1364.
[3]MERMUYS K,VANHOENACKER P K,ROSKAMS T,et al.Epithelioid hemangioendothelioma of the liver:radiologic-pathologic correlation[J].Abdom Imaging,2004,29(2):221-223.
[4]MILLER W J,DODD G D 3RD,FEDERLE M P,et al.Epithelioid hemangioendothelioma of the liver:imaging findings with pathologic correlation[J].Am J Roentgenol,1992,159(1):53-57.
[5]WEISS S W,ENZINGER F M.Epithelioid hemangioendothelioma:a vascular tumor often mistaken for a carcinoma[J].Cancer,1982,50(5):970-981.
[6]MAKHLOUF H R,ISHAK K G,GOODMAN Z D.Epithelioid hemangioendothelioma of the liver:a clinicopathologic study of 137 cases[J].Cancer,1999,85(3):562-582.
[7]OTROCK Z K,AL-KUTOUBI A,KATTAR M M,et al.Spontaneous complete regression of hepatic epithelioid haemangioendothelioma[J].Lancet Oncol,7(5):439-441.
[8]EKFORS TO,JOENSUU K,TOIVIO I,et al.Fatal epithelioid haemangioendothelioma presenting in the lung and liver[J].Virchows Arch A Pathol Anat Histopathol,1986,410(1):9-16.
[9]UCHIMURA K,NAKAMUTA M,OSOEGAWA M,et al.Hepatic epithelioid hemangioendothelioma[J].J Clin Gastroenterol,2001,32(5):431-434.
[10]ECONOMOPOULOS N,KELEKIS N L,ARGENTOS S,et al.Bright-dark ring sign in MR imaging of hepatic epithelioid hemangioendothelioma[J].J Magn Reson Imaging,2008,27(4):908-912.
[11]ALOMARI AI.The lollipop sign:a new cross-sectional sign of hepatic epithelioid hemangioendothelioma[J].Eur J Radiol,2006,59(3):460-464.
[2]LYBURN I D,TORREGGIANI W C,HARRIS A C,et al.Hepatic epithelioid hemangioendothelioma:sonographic,CT,and MR imaging appearances[J].Am J Roentgenol,2003,180(5):1359-1364.
[3]MERMUYS K,VANHOENACKER P K,ROSKAMS T,et al.Epithelioid hemangioendothelioma of the liver:radiologic-pathologic correlation[J].Abdom Imaging,2004,29(2):221-223.
[4]MILLER W J,DODD G D 3RD,FEDERLE M P,et al.Epithelioid hemangioendothelioma of the liver:imaging findings with pathologic correlation[J].Am J Roentgenol,1992,159(1):53-57.
[5]WEISS S W,ENZINGER F M.Epithelioid hemangioendothelioma:a vascular tumor often mistaken for a carcinoma[J].Cancer,1982,50(5):970-981.
[6]MAKHLOUF H R,ISHAK K G,GOODMAN Z D.Epithelioid hemangioendothelioma of the liver:a clinicopathologic study of 137 cases[J].Cancer,1999,85(3):562-582.
[7]OTROCK Z K,AL-KUTOUBI A,KATTAR M M,et al.Spontaneous complete regression of hepatic epithelioid haemangioendothelioma[J].Lancet Oncol,7(5):439-441.
[8]EKFORS TO,JOENSUU K,TOIVIO I,et al.Fatal epithelioid haemangioendothelioma presenting in the lung and liver[J].Virchows Arch A Pathol Anat Histopathol,1986,410(1):9-16.
[9]UCHIMURA K,NAKAMUTA M,OSOEGAWA M,et al.Hepatic epithelioid hemangioendothelioma[J].J Clin Gastroenterol,2001,32(5):431-434.
[10]ECONOMOPOULOS N,KELEKIS N L,ARGENTOS S,et al.Bright-dark ring sign in MR imaging of hepatic epithelioid hemangioendothelioma[J].J Magn Reson Imaging,2008,27(4):908-912.
[11]ALOMARI AI.The lollipop sign:a new cross-sectional sign of hepatic epithelioid hemangioendothelioma[J].Eur J Radiol,2006,59(3):460-464.