上皮样血管平滑肌脂肪瘤的病理诊断与临床分析

目的探讨上皮样血管平滑肌脂肪瘤的临床病理特征及鉴别诊断。方法回顾性分析5例上皮样血管平滑肌脂肪瘤的临床和病理改变状况,以及组织形态和免疫组化标记物的表达特点。结果组织学显示上皮样血管平滑肌脂肪瘤的肿瘤细胞多呈巢状或片状排列,体积大,多形性或梭形,有丰富的嗜酸性颗粒状胞浆,细胞核较大,核仁明显呈空泡状,可见特征性的血管样结构以及上皮样肿瘤细胞围绕血管呈袖套样排列,肿瘤成分缺少脂肪组织,免疫组织化学染色示肿瘤细胞HMB-45,Melan-A弥漫阳性。结论上皮样血管平滑肌脂肪瘤是一种具有恶性潜能的间叶源性肿瘤,免疫组化对鉴别有重要意义。     

肾上皮样血管平滑肌脂肪瘤临床特点分析

目的 探讨肾上皮样血管平滑肌脂肪瘤的临床及病理特点.方法 肾上皮样血管平滑肌脂肪瘤患者6例.单发4例,多发2例.肿瘤直径平均9(5～14)cm.有结节硬化家族史1例.CT检查发现肿瘤内含脂肪组织(CT值-30～-120 HU)3例,诊断为肾血管平滑肌脂肪瘤;3例瘤组织CT值30～55 HU,增强扫描CT值70～110 HU.行肿瘤剜除术3例,行根治性肾切除术3例.结果 3例行肿瘤剜除术者肿瘤完整剜除,2例出现肾盂破损,行肾盂修补及留置双J管.其中1例双肾肿瘤者,2个月后手术剜除对侧肿瘤.3例根治性肾切除者完整切除肾脏、肾周脂肪组织及肿大淋巴结.病理检查:镜下肿瘤以上皮样细胞为主,并出现多形、异形核,有明显的上皮样分化,浸润破坏性生长,瘤组织广泛坏死.免疫组化染色HMB45(+++),EMA、CK(-).病理诊断均为肾上皮样血管平滑肌脂肪瘤.平均随访26(10～44)个月,肿瘤无复发及转移5例;1例根治术后18个月出现双肺转移,10个月后死亡.结论 肾上皮样血管平滑肌脂肪瘤多为良性,部分缺乏脂肪组织,主要由单核或多核的上皮样细胞构成,免疫组化染色HMB45强阳性为特征表现,具有潜在恶性倾向,可发生转移;CT检查易误诊为肾癌;手术为主要治疗方法.     

肾上皮样血管平滑肌脂肪瘤诊治体会(附1例报告及文献复习)

上皮样血管平滑肌脂肪瘤(EAML)是血管平滑肌脂肪瘤(AML)的一个亚型,主要发生在肾脏,具有恶性潜能,临床少见。我院2010年收治1例肾EAML患者,现结合文献复习报告如下。     

肝上皮样血管平滑肌脂肪瘤临床病理诊断

上皮样血管平滑肌脂肪瘤(epithelioid angiomyolipoma,EAML)是血管平滑肌脂肪瘤(angiomyolipoma,AML)的罕见亚型,主要发生于肾脏,但可偶见于肾外,如肝、肺、淋巴结、腹膜后等部位[1],我们近期遇到3例肝EAML,报告如下.     

肝上皮样血管平滑肌脂肪瘤临床病理诊断

上皮样血管平滑肌脂肪瘤(epithelioid angiomyolipoma,EAML)是血管平滑肌脂肪瘤(angiomyolipoma,AML)的罕见亚型,主要发生于肾脏,但可偶见于肾外,如肝、肺、淋巴结、腹膜后等部位[1],我们近期遇到3例肝EAML,报告如下.     

肝上皮样血管平滑肌脂肪瘤临床病理诊断

上皮样血管平滑肌脂肪瘤(epithelioid angiomyolipoma,EAML)是血管平滑肌脂肪瘤(angiomyolipoma,AML)的罕见亚型,主要发生于肾脏,但可偶见于肾外,如肝、肺、淋巴结、腹膜后等部位[1],我们近期遇到3例肝EAML,报告如下.     

肾血管平滑肌脂肪瘤的诊治特点

肾血管平滑肌脂肪瘤是较为常见的肾脏肿瘤,约占所有肾肿瘤的10％,其总发病率约为0．3％[1].一般来说,肾血管平滑肌脂肪瘤属于良性病变,其典型结构包括厚壁且紊乱的血管、平滑肌以及成熟脂肪组织,人们一度认为这是错构瘤的一种形态,但最近的证据显示其是单克隆而不是多克隆来源,它是来源于血管周围上皮样细胞,属于血管周上皮样肿瘤这一类别[2-3]。     

MSCT对肾血管平滑肌脂肪瘤的诊断价值

目的：探讨多层螺旋CT（MSCT）在肾血管平滑肌脂肪瘤诊断中的价值。方法：对手术证实的38例肾血管平滑肌脂肪瘤的术前多层螺旋CT资料进行回顾性分析,并在工作站行MPR、MIP图像重建,分析肾血管平滑肌脂肪瘤的MSCT表现,并与手术病理结果对照。结果：38例肾血管平滑肌脂肪瘤中,CT诊断血管平滑肌脂肪瘤的病灶被手术病理证实正确诊断的有32例,另外6例被病理证实为上皮样血管平滑肌脂肪瘤的病灶,其中3例CT误诊为肾癌,后期的3例被正确诊断。总诊断准确率达92.1%（35/38）。32例的血管平滑肌脂肪瘤CT平扫呈等、低、高低混杂密度的,其中31例显示有脂肪密度,多期增强扫描该31例皮质期肿块呈均匀或不均匀强化,但低于肾皮髓质强化,脂肪、坏死囊变、出血区无强化,分泌期肿块持续强化;1例整个病灶显示为高密度出血灶,多期增强扫描无明显异常强化;6例上皮样血管平滑肌脂肪瘤CT平扫呈均匀等密度,多期增强扫描显示皮质期明显强化,分泌期强化减弱。MIP显示其中31例血管平滑肌脂肪瘤的肿内迂曲、增粗的血管,其中1例无明显增粗迂曲血管影;6例上皮样血管平滑肌脂肪瘤内显示细小血管。结论：多层螺旋CT及多期增强扫描能对术前正确诊断肾血管平滑肌脂肪瘤有重要价值,但对上皮样血管平滑肌脂肪瘤的诊断尚需结合临床和实验室检查,甚至需要穿刺活检。     

腹腔镜下微波消融剜除术治疗肾脏肿瘤的疗效分析

目的 探讨腹腔镜下微波消融肾肿瘤剜除术治疗肾脏肿瘤的疗效与安全性.方法 本组84例肾肿瘤患者,予以腹腔镜下辅助微波消融,尔后手术剜除肾肿瘤,记录术中出血量、手术时间,观察术中、术后并发症及疗效与安全性.结果 84例患者手术均顺利完成,术中均未阻断肾动脉,无术中大出血或中转开放手术,出血量为50~350 ml,手术时间为70~120 min.术后病理诊断为肾透明细胞癌60例,血管平滑肌脂肪瘤16例,嗜酸性细胞瘤4例,嫌色细胞癌2例,乳头状肾细胞癌2例.术中血管平滑肌脂肪瘤普遍出血较多.术后血管平滑肌脂肪瘤出血1例.全部病例随访12个月,无肿瘤复发、远处转移或死亡病例.结论 腹腔镜下微波消融肾肿瘤剜除术是一种安全有效的保留肾单位的手术方式,具有出血量少、手术时间短、术后并发症少等优点.     

肝血管平滑肌脂肪瘤的临床病理分析

目的探讨肝血管平滑肌脂肪瘤的临床病理特点、诊断及鉴别诊断。方法对3例肝血管平滑肌脂肪瘤患者有关病理检查结果进行回顾性分析。结果肿瘤位于肝右叶2例，肝左叶1例。肿瘤直径为2～10cm．平均6．2cm。3例肿瘤内均见平滑肌、脂肪、畸形厚壁血管．但未见髓外造血灶。对黑色素瘤(HMB45)、结合蛋白(desmin)及肌动蛋白(actin)检查均呈阳性反应。术后随访6～36个月．未见肿瘤复发。结论肝血管平滑肌脂肪瘤由3种成分组成，病理形态变化多样，必须与多种肝肿瘤相鉴别。平滑肌细胞HMB45表达呈强阳性反应是诊断肝血管平滑肌脂肪瘤较可靠的依据。     

Epithelioid angiomyolipoma of the kidney

Epithelioid angiomyolipoma is a recently recognized variant of angiomyolipoma, which is characterized by the presence of polygonal cells with densely eosinophilic cytoplasm and varying degrees of nuclear atypia. Only a relatively small number of cases of epithelioid angiomyolipoma of the kidney have been reported in the literature. We report a case of epithelioid angiomyolipoma of the kidney that occurred in a 38-year-old woman. The tumor was composed of diffuse sheets of epithelioid cells, adipocytes and only scattered thick-walled blood vessels. The epithelioid cells had pleomorphic and hyperchromatic nuclei with densely eosinophilic cytoplasm. Hemorrhage, necrotic foci and clusters of foamy macrophages were present. HMB-45, CD117 (c-kit) and CD68 were detected in the epithelioid cells. There was no expression of cytokeratin, epithelial membrane antigen or desmin. The patient showed no evidence of recurrence or metastatic disease 9 months after nephrectomy.     

转移性恶性肾血管平滑肌脂肪瘤三例报告

目的 探讨转移性恶性肾血管平滑肌脂肪瘤(AML)的诊断和治疗. 方法转移性恶性AML患者3例.男1例,女2例.年龄分别为36、55和37岁.主诉为持续性患侧腰痛1～6个月.2例B超、CT检查发现患肾实性占位.1例B超见肾肿块内不均匀高回声光团,CT测得脂肪负值信号.2例行根治性肾切除术,1例行肾切除术.3例均未放化疗. 结果 肿块切面灰红、灰黄,质软.病理镜下观察:2例瘤细胞呈上皮样,核有异型,伴坏死;1例大多区域为梭形平滑肌,脂肪细胞和血管混杂生长,部分区域细胞呈上皮样.免疫组化结果 均为VM(+),HMIM5(+),Melan-A(+),S-100(-),CK(-).3例病理诊断均为恶性肾AML,2例为上皮样型,1例为经典型、部分上皮样型.1例术后7年出现肺转移,行肺叶切除加清扫,随访12年无瘤生存.1例术后6年复发,行肿块切除术;术后lO年发现肝、后腹膜转移,再行肿块部分切除,随访12年带瘤生存.1例术后4个月出现肝、后腹膜转移,行肿块部分切除,3个月后失访. 结论 恶性肾AML罕见,诊断依靠病理和免疫组化检查.治疗方法无特殊.     

Variants of renal angiomyolipoma closely simulating renal cell carcinoma: difficulties in the histological diagnosis

Renal angiomyolipoma is considered to be a benign renal tumor composed of atypical blood vessels, smooth muscles and fat cells. We report 2 cases of unilateral renal angiomyolipoma. In both cases, our preoperative diagnosis was renal cell carcinoma, because no low density area compatible with fat tissue was noted in the tumors on radiographic evaluation. Through histological examination, both tumors proved to be angiomyolipomas mainly composed of epithelioid cells in 1 case, and spindle-shaped smooth muscle cells mimicking a leiomyoma in the other case. Both patients are well showing no evidence of metastases 16 and 14 months after nephrectomy, respectively.     

Renal angiomyolipoma with malignant transformation, simultaneous occurrence with malignity and other complex clinical situations

Renal angiomyolipoma is a benign kidney tumor, which is characterized by the presence of mature or immature fat tissue, thick-walled blood vessels, and smooth muscles. However, there is a rare possibility of transformation to a malignancy. This transformation could be toward sarcoma. And also angiomyolipoma could be associated with renal adenocarcinoma in the same kidney. The aim of this study is to show the association of angiomyolipoma with complex clinical situations such as malign transformation, simultaneous occurrence with adenocarcinoma, bilateral tumors with tuberous sclerosis or Wunderlich Syndrome. Findings of clinical presentation, pathological evaluation, urography, ultrasonography, and computerized tomography of 19 patients who were diagnosed angiomyolipoma in our clinic during 1994–2003 were examined retrospectively. Our records indicated that radical nephrectomy was performed in three cases of angiomyolipoma in which the differential diagnosis from adenocarcinoma could not be made and in another case of angiomyolipoma, which was associated with adenocarcinoma. Simple nephrectomy was performed in four symptomatic angiomyolipoma cases with tumor size larger than 4 cm and partial nephrectomy in another case. In one case, renal angiomyolipoma was associated with adenocarcinoma in the same kidney. In one case, post-operative recurrence of angiomyolipoma developed 7 months after nephrectomy. This patient was diagnosed low-grade leiomyosarcoma. Angiomyolipoma is regarded as a benign tumor of the kidney; however, it may also show aggressive behaviors and rarely transform to sarcoma. It may also exist in the same kidney along with adenocarcinoma or sarcoma.     

Malignant epithelioid angiomyolipoma ('sarcoma ex angiomyolipoma') of the kidney: a case report and review of the literature

Malignant epithelioid angiomyolipoma is a recently described rare tumor of the kidney. Its existence has been questioned, however, on the basis of incomplete evidence of malignant behavior, the absence of an associated classic angiomyolipoma component, or the absence of immunoreactivity for HMB-45 in some cases. We describe a case that was HMB-45-positive and arose in association with a classic angiomyolipoma. The patient was treated with a partial nephrectomy. Three years later, she developed rapidly enlarging liver nodules. A fine-needle aspiration of the liver confirmed the presence of pleomorphic epithelioid cells morphologically and immunohistochemically identical to those comprising the primary renal tumor. After two cycles of treatment with doxorubicin, there was a 50% reduction in the size of the tumors with marked improvement in performance status. We believe this case confirms the existence of a malignant epithelioid angiomyolipoma.     

肾上皮样血管平滑肌脂肪瘤3例临床病理观察

目的：探讨肾上皮样血管平滑肌脂肪瘤（Angiomyolipoma，AML）的临床病理特征及鉴别诊断依据。方法：回顾性分析3例肾上皮样AML的临床和病理改变状况，以及组织形态及免疫标记物的表达特点。结果：3例术前影像学检查均考虑为肾癌，镜下肿瘤内均缺乏脂肪组织，可见特征性的血管结构以及上皮样肿瘤细胞围绕血管呈袖套样排列，免疫组织化学染色示肿瘤细胞HMB45或Melan-A弥漫阳性，SMA阳性表达不定。结论：肾上皮样AML是一种少见的具有恶性潜能的肿瘤，诊断中需要注意与肾细胞癌相鉴别。免疫组织化学染色是诊断肾上皮样AML的一项必不可少的技术手段。     

Surgeon-controlled robotic partial nephrectomy for a rare renal epithelioid angiomyolipoma using near-infrared fluorescence imaging using indocyanine green dye: A case report and literature review

Renal epithelioid angiomyolipoma (E-AML) is a rare variant of angiomyolipoma (AML). It is a mesenchymal tumour believed to originate from the perivascular epithelioid cell (PEC). Unlike conventional AML which are benign, E-AML has a rare aggressive behaviour. Conventional AML is typically triphasic containing adipose tissue, smooth muscle and dystrophic vessels in variable proportions, while E-AML are generally composed of plump spindled and polygonal-shaped “epithelioid cells” showing clear or eosinophilic cytoplasm and occasional pleomorphic multinucleated giant cells. E-AML can be misdiagnosed as renal cell carcinoma (RCC) when these “epithelioid cells” show clearing. Only a small number of cases of E-AML have been reported with the standard treatment being radical or partial nephrectomy. We report the first case report of a surgeon-controlled robotic partial nephrectomy using a near-infrared fluorescence imaging using indocyanine green dye on a 25-year-old woman with a T1B (6.6 cm) right renal mass. The final pathology revealed the diagnosis of E-AML. There was no recurrence and metastases after the 6-month follow-up.     

Hepatic angiomyolipoma developing during the follow-up of ulcerative colitis: Report of a case and review of the literature

Hepatic angiomyolipoma is a rare tumor composed of spindle-shaped and epithelioid smooth muscle cells, adipose tissue, and proliferating blood vessels. We report the first documented case of this tumor developing in a patient with ulcerative colitis. A solitary tumor (7.5×7.5×7cm) was detected in the left lateral segment of the liver and a left hepatic lobectomy was performed. The diagnosis of angiomyolipoma was confirmed by a pathological examination. We also review the literature on previously reported cases of hepatic angiomyolipoma.     

The limit in differential diagnosis of renal angiomyolipoma from renal cell carcinoma on computerized tomography: a case report

Recent reports have described that the accurate preoperative diagnosis of renal angiomyolipoma is possible with computerized tomography (CT). There are, however, some cases of angiomyolipoma which are indistinguishable from renal cell carcinoma on CT. Herein we report a case of renal angiomyolipoma which CT failed to reveal preoperatively. In a 71-year-old Japanese male, a left renal tumor was incidentally suspected on excretory urogram. Then CT demonstrated a homogeneous mass in the upper part of the left kidney, 6 cm in diameter, which showed a positive attenuation coefficient, slightly denser than renal parenchyma. Selective left renal arteriogram showed hypervascularity without aneurysmal formation or arteriovenous fistula. These radiologic findings strongly suggested renal cell carcinoma and radical left nephrectomy was performed. Histological examination confirmed angiomyolipoma composed mainly of smooth muscle with thick-walled vessels and immature fat cells. We reviewed the literature and discussed the computerized tomographic-pathologic correlation of angiomyolipoma.