Epithelioid angiomyolipoma of the liver: CT and MRI features

The purpose of our study was to retrospectively evaluate the computed tomography (CT)/magnetic resonance imaging features of epithelioid angiomyolipoma of the liver (Epi-HAML), with pathology as a reference. We reviewed the CT/MRI findings of six lesions of Epi-HAML and found absence of adipose tissue in the lesions. In addition, recognizing the imaging features of no capsule, and hypervasularity with central punctiform or filiform vessels as a characteristic enhancement may distinguish Epi-HAML from other hepatic tumors.     

Hepatic angiomyolipoma: identification of an efferent vessel as a hepatic vein by contrast-enhanced harmonic sonography

Two cases of hepatic angiomyolipoma were studied by contrast-enhanced harmonic sonography. The special tumor hemodynamics, namely the efferent blood flow of the hepatic angiomyolipoma draining into the hepatic vein, were clearly shown on harmonic imaging, and they corresponded well with those seen on angiography and computed tomography during angiography. Benign hepatic tumors were diagnosed preoperatively in both cases according to the hemodynamic findings. Hepatic angiomyolipoma was finally identified histologically. The special tumor hemodynamics might be one of the important characteristics of hepatic angiomyolipoma. Contrast-enhanced harmonic sonography is useful for the detection of special tumor hemodynamics and may facilitate the differential diagnosis from other hepatic tumors, especially malignant liver tumors.     

Clinical and Contrast-enhanced Ultrasound Characteristics of Epithelioid and Classic Hepatic Angiomyolipoma: Comparison With Alpha-fetoprotein–negative Hepatocellular Carcinoma

Hepatic angiomyolipoma (HAML) comprises epithelioid angiomyolipoma (EAML) and classic hepatic angiomyolipoma (CAML). The imaging appearance of HAML varies widely, and EAML is more easily misdiagnosed as hepatocellular carcinoma (HCC) than as CAML. The clinical and contrast-enhanced ultrasound (CEUS) features of CAML, EAML and HCC with negative alpha-fetoprotein protein expression (HCC[AFP–]) were retrospectively reviewed. The hyper-vascular type was more commonly found in CAML and EAML lesions than in HCC lesions. Most lesions were hyper-enhanced in the arterial phase. CAMLs showed prolonged hyper-enhancement or iso-enhancement during the portal and late phases on CEUS, making them easily distinguishable from HCC(AFP–). Some EAML lesions (41.7%) were hypo-echoic, similar to HCC(AFP–). However, the hypo-enhancement of EAML lesions occurred later than that of HCC(AFP–) lesions. Thus, our findings may be useful in distinguishing among these lesions to improve diagnostic accuracy.     

Rare case of angiomyolipoma of the larynx

We report a rare case of angiomyolipoma (AML) of the larynx. AML belongs to the family of perivascular epithelioid cell tumors (PEComas). We review the literature on PEComas and describe differences in immunohistochemical findings between renal AML and AML in the head and neck region.     

Hemodynamic characterization of hepatic angiomyolipoma with least amount of fat evaluated by contrast-enhanced magnetic resonance angiography

The purpose of our study was to describe contrast-enhanced magnetic resonance angiography (MRA) findings of hepatic angiomyolipoma (AML) with less lipocyte. We reviewed the MRA findings of five resected lesions of hepatic AML with less lipocyte specimens and found that the efferent vessels of hepatic AMLs were the hepatic veins. These findings corresponded well with those seen on conventional contrast-enhanced dynamic CT scans. This hemodynamic finding may be an important characterization of hepatic AML with less lipocyte, and could distinguish AML from other benign and malignant hepatic tumors.     

超声造影对提高肾错构瘤诊断率的价值

目的探讨超声造影在肾错构瘤诊断中的价值。方法对37例患者37个经手术病理证实的肾错构瘤常规超声及超声造影声像图进行回顾性分析。常规超声观察肿瘤大小、边界、内部回声、有无彩色血流信号。超声造影观察病灶的增强时相和方式,包括起始时间、达峰值时间、消退时间及病灶内部结构的增强表现。结果常规超声显示肿块最大直径范围1.7～28.0cm,高回声团块19个,低回声为主团块11个,混合回声6个,无回声1个。19个肿块发现较丰富或丰富彩色血流信号,16个显示周边或内部少量血流信号,2例未见血流信号。术前常规超声诊断符合率为59.5%(22/37)。37个肿块超声造影平均增强起始时间为(13.57±3.34)s,达峰时间为(20.27±4.27)s,开始消退时间为(28.92±5.16)s。皮质期33个病灶呈缓慢向心增强,4个同步增强;达峰值呈高增强15个,等增强14个,低增强8个。其中28例均匀增强。实质期同步消退11个,缓慢消退19个,快速消退7个,发现假包膜2例。联合缓慢向心增强和达峰值均匀等增强或高增强的诊断符合率为75.7%(28/37)。结论造影时缓慢向心增强和均匀等增强或高增强是肾错构瘤的主要表现。     

Giant renal angiomyolipoma with fatal hemorrhage due to a large pseudoaneurysm

We report a case of giant renal angiomyolipoma (AML) with a large pseudoaneurysm causing fatal hemorrhage. A 60-year-old woman presented with right-sided abdominal pain. Sonographic examination revealed a large hyperechoic renal mass. CT examination of the abdomen revealed a 15-cm AML of the right kidney with a large hemorrhagic area within it. Subsequent color Doppler examination revealed a 2.9-cm pseudoaneurysm arising from the upper segmental branch of the right renal artery. The patient, who was about to undergo transcatheter embolization of the pseudoaneurysm, died from cardio-respiratory arrest. The autopsy findings confirmed a large right renal AML containing a thrombosed pseudoaneurysm and a large perinephric hematoma.     

肝血管平滑肌脂肪瘤的超声造影表现

目的 探讨肝血管平滑肌脂肪瘤(angiomyolipoma,AML)的超声造影表现.方法 回顾性分析9例经病理确诊的肝AML患者共10个病灶的常规超声和超声造影表现.超声造影采用SonoVue和对比脉冲序列成像技术.结果 常规超声7个病灶呈现混合回声,即明显高回声伴片状低回声;3个病灶呈明显高回声.彩色多普勒超声检查所有病灶均测得动脉血供,且血供丰富或较丰富或稀少,阻力指数平均0.53±0.10(0.48～0.62).超声造影显示9个病灶动脉期均呈高增强;门脉期5个为等增强,1个为高增强,3个为低增强;延迟期6个为等增强,3个为低增强.1个病灶三期均为低增强.结论 超声造影上肝AML的增强表现具有一定特征性,可用于提示诊断,但仍需积累更多经验.     

CT诊断肾少脂肪血管平滑肌脂肪瘤与透明细胞癌的临床分析

目的研究CT诊断肾少脂肪血管平滑肌脂肪瘤(AML)与透明细胞癌(CRCC)的临床特征,提高诊断的准确率。方法选择AML与CRCC患者各30例为研究对象,分为AML组和CRCC组。回顾性两组患者的CT检查情况以及相关特征指标。结果两组在肿瘤密度、强化程度方面无显著性差异,强化类型上AML组均匀强化显著多于CRCC组,差异有统计学意义(P0.05)。且AML组患者在病灶突出比、皮质掀起征两个方面均大于CRCC组,差异有统计学意义(P0.05)。结论 AML组与CRCC组在CT中表现出的差异较大,AML无论在肿瘤均匀性、病灶突出比还是皮质掀起证都明显大于CRCC,因此应用CT能够准确地鉴别二者。     

肝脏血管平滑肌脂肪瘤的超声诊断

报道４例经病理证实的肝脏血管平滑肌脂肪瘤。超声表现为边界清楚，强回声富血供的实性肿块，其后方回声增强，其中３例肿瘤后方的肝表面或膈肌向外轻度移位。对于典型的肝脏血管平滑肌脂肪瘤超声结合其它影像学检查术前可以确诊。     

Unusual sonographic appearance of a huge angiomyolipoma of the liver

Hepatic angiomyolipoma is a rare, benign, hepatic mesenchymal neoplasm. We report a case of an extremely large hepatic angiomyolipoma in a 58‐year‐old woman with unusual sonographic features. The tumor was well demarcated from the surrounding liver and showed a heterogeneous cystic appearance with a thick echogenic rim. Color Doppler showed blood flow in the echogenic rim and around the lesion. Contrast sonography demonstrated strong enhancement in the solid rim from early arterial phase to the late phase and no enhancement in the central cystic part of the lesion. Spiral CT showed a well‐defined huge heterogeneous cystic mass with fatty density. At surgery the tumor was soft and encapsulated with a thin, smooth membrane. The cut surface of the tumor revealed a cystic appearance with necrotic tissue. The diagnosis of hepatic angiomyolipoma was confirmed by immunohistochemistry. © 2010 Wiley Periodicals, Inc. J Clin Ultrasound, 2010     

肾错构瘤破裂出血的超声图像分析

目的 探讨肾错构瘤破裂出血的超声诊断。方法 回顾性分析了1994—2002年9例肾错构瘤破裂出血的超声图像。结果 9例肾错构瘤3例双侧，6例单侧，4例多发，5例单发。8例肾错构瘤见肾内外高回声光团相连，肾周见无回声区和混合性回声区积液。1例为肾错构瘤内无回声区。结论 超声对肾错构瘤破裂出血的术前诊断具有重要价值。     

腹部超声检查在结节性硬化症中的应用价值

目的分析结节硬化症(TSC)的腹部超声表现,提高对该病的认识及诊断准确率。方法回顾性分析临床确诊的96例TSC患者腹部超声表现。结果 96例患者中无病变组31例,病变组65例,无病变组和病变组在性别及年龄的差异具有统计学意义(P<0.05)。病变组65例中,共64例有肾脏病变,32例有肝脏病变,其中31例肾脏合并肝脏病变。64例肾脏病变共包括血管平滑肌脂肪瘤(AML)62例、囊肿15例、肾癌1例,其中13例AML合并囊肿,1例AML合并肾癌;32例肝脏病变共包括AML 22例、血管瘤2例、囊肿13例、钙化2例,其中7例AML合并囊肿。结论腹部超声检查能够发现TSC腹部相关病变,有助于该病诊断,是其重要影像学检查方法。     

超声对非典型肾错构瘤的诊断价值

目的探讨非典型肾错构瘤超声分型及其与病理的关系。方法分析44例非典型声像型肾错构瘤的声像图分型,并与病理结果对照。结果44例非典型肾错构瘤,肿瘤呈无回声型1例,直径在4—10cm,病理提示瘤体内大部分坏死出血;低回声型4例,直径〈4cm,病理提示瘤体以平滑肌为主;混合回声型39例,3例直径在4—10cm,36例直径〉10cm,病理提示瘤体内部分坏死出血。超声诊断肾错构瘤37例,诊断符合率为84．1％,误诊为肾癌7例。结论超声对非典型肾错构瘤的诊断有较高价值。     

Hepatic Angiomyolipomas: Ultrasonic Characteristics of 25 Patients from a Single Center

Twenty-five pathologically proven hepatic angiomyolipomas (AMLs) were included in the study. Ultrasonic features of hepatic AMLs were reviewed. Three types of echogenicity were observed on ultrasound examination: (i) strong hyper-echogenicity, (ii) moderate hyper-echogenicity and (iii) hypo-echogenicity. Vascular signals within tumors could be detected in 22 (88.00%) tumors as multiple punctiform, filiform or dendriform signals by color Doppler flow imaging. Based on the enhancement patterns in the arterial, portal and late phases, the features of hepatic AMLs on contrast-enhanced ultrasound were divided into four subtypes: (i) “fast in slow out” (68.00%, n = 17); (ii) “fast in same out” (16%, n = 4); (iii) “fast in fast out” (12.00%, n = 3); and (iv) “fast in uneven out” (4.00%, n = 1). Contrast-enhanced ultrasound diagnosed 22 (88.00%) tumors as benign tumors and 13 (52.00%) as hepatic AMLs. Four cases were misdiagnosed as hepatic hemangioma, five cases as focal nodular hyperplasia (total = 36.00%). The rate of correct diagnosis of hepatic AMLs increased significantly from 24.00% for ultrasound alone to 52.00% for contrast-enhanced ultrasound. Therefore, information obtained from ultrasound, color Doppler flow imaging and contrast-enhanced ultrasound should be combined to improve diagnosis.     

超声诊断巨大肾血管平滑肌脂肪瘤

目的 探讨巨大肾血管平滑肌脂肪瘤(最大径大于5 cm)的声像图特点. 方法 回顾性分析手术病理证实的17个巨大肾血管平滑肌脂肪瘤的超声表现,包括病变大小、边界、位置、内部回声及血流特点. 结果 采用超声检查,11个准确定位(其中9个正确诊断为RAML),5个定位不清,1个误诊为肾上腺占位;声像图表现为边界清晰的高回声、低回声、中等回声或高低回声相间的混合性回声.较大的高回声、大多数混合性回声及所有低回声病灶内可探及程度不等的血流信号,其声像图表现类型与病灶内脂肪、平滑肌和血管的组成和分布密切相关. 结论 巨大肾血管平滑肌脂肪瘤具有特征性的声像图表现,超声结合其他影像学检查,对多数病灶可于术前明确诊断.     

Sonographic appearance of a cholesterol granuloma within a renal epithelioid angiomyolipoma

We present the sonographic characteristics of a rare case of cholesterol granuloma within a renal epithelioid angiomyolipoma (AML) in a 57-year-old woman. Sonography showed acoustic shadowing distal to the central region of the AML behind a hyperechoic cholesterol, which was hypoattenuating on CT with no contrast enhancement. Microscopic examination confirmed that this central area was a cholesterol granuloma. The patient was treated successfully with radical nephrectomy.     

后腹腔镜下微波消融治疗肾错构瘤

目的探讨后腹腔镜下微波消融治疗肾错构瘤的合理性、安全性、临床疗效和并发症。方法2007年7月至2008年6月,对6例肾错构瘤患者进行后腹腔镜直视下微波消融治疗。结果所有患者接受3～5个点的微波消融（50W,每个点3～8min）,手术平均时间55min,均无明显手术并发症。术后1～3个月6例患者均行CT复查,均见肿瘤呈消融术后改变。结论后腹腔镜下微波消融治疗肾错构瘤是可行的,手术时间短,并发症少,并可达到手术预期目的,是一种安全、有效的肾错构瘤微创治疗方法。     

少脂肪肾错构瘤的超声造影表现

目的：探讨超声造影在少脂肪肾错构瘤中的表现。方法：对11例11个经手术病理证实的少脂肪肾错构瘤的常规超声及超声造影声像图进行回顾分析。常规超声检查观察肾占位大小、回声、边界、有无彩色血流信号。超声造影观察病灶的增强时相和方式,包括起始时间、达峰值时间、消退时间及病灶内部结构的增强表现。结果：11个肿块最大直径范围2．4～15．3cm,常规超声显示低回声为主不均团块9个,囊实性团块2个,均发现较丰富或丰富彩色血流信号,术前诊断准确率仅36．4％（4／11）。超声造影显示皮质期11个病灶均缓慢增强、向心型填充;达峰值时均呈高回声或等回声,9例均匀增强;实质期同步消退4个,缓慢消退5个,快速消退2个,发现类似“假包膜”征2例。结论：造影时缓慢向心增强和达峰值均匀增强是低回声少脂肪肾错构瘤的主要表现。     

能量多普勒和灰阶超声对肾实体占位的诊断及鉴别诊断

目的　探讨肾实体肿瘤的灰阶超声特征及其能量多普勒血供模式 ,并评价二者联合应用的诊断及鉴别诊断价值。方法　用灰阶超声及能量多普勒超声评价 4 2例肾实体肿瘤。在灰阶超声图上 ,观察肿块回声、均匀性、无回声边、肿瘤内无回声区及后方衰减。在能量多普勒超声上 ,肿块的血管分布被分成 5种模式。结果　 4 2例肾实体肿瘤 ,肾细胞癌 (RCC) 33例 ,肾血管平滑肌脂肪瘤 (AML ) 9例。在灰阶超声上 ,2 4例 (73% ) RCC可见无回声边和 /或肿瘤内无回声区 ;2 6例 (79% ) RCC肿块回声不均匀。 1例 AML (11% )出现声衰减。肿块回声强度不能提示病理诊断。在能量多普勒超声上 ,33例 RCC中 ,模式 3(边缘血流型 ) 4例 ,模式 4 (混合性边缘及穿入血流型 ) 2 9例。 9例 AML中 ,模式 4血流 1例 ,模式 0 (无血流信号型 ) 5例 ,模式 2 (穿入性血流型 ) 3例。在本组 RCC及 AML中均未见模式 1(肿瘤内局限性血流信号型 )。模式 0、模式 2对 AML有特异性。与单独灰阶超声 (6 0 % )及单独能量多普勒超声 (19% )的诊断率比较 ,二者联合诊断率 (79% )明显提高。结论　无回声边及肿瘤内无回声区提示 RCC的诊断。在灰阶超声发现的基础上 ,能量多普勒超声血管分布为 RCC及 AML的鉴别诊断提供了重要的依据