范文一：肝血管平滑肌脂肪瘤一例

DOC格式论文～方便的论制修改论您减

肝血管平滑肌脂肪瘤一例

;作者论位论论,:___________: ___________: ___________

作者,汲武　论朝　论新　田论天　王晨宇　姜洪池广阳刘

【论论论】 血管肌瘤?脂肪瘤?肝论瘤

1　病例介论

患者男～54论。于2008年3月因格论论论论肝论占位我院住院论论～主体来

论无明论不适。入院论体:皮论膜无染～心、肺未论常～腹平论～肝脾巩黄异

肋下未及～右上腹论痛;-,～反跳痛;-,。论助论论,甲胎蛋白;alpha fetoprotein, AFP,、癌胚抗原;cacinoembryonic antigen,

CEA,、CA199 均正常。肝炎指论及肝功能均正常～B超示肝右可论叶一大小论66 mm×57 mm的略高回光论～论界～肝右论系密声清与静脉

切～部回不均～可论不论论低回～但未论血流信～论主内声极匀声号静脉

干呈向肝性～肝管未论论论。增强内胆CT示肝右可论叶7 cm×6 cm大小低密度论论～密度不均～论期病论论论斑片强化～论期及延论匀脉灶状脉

期病论论明论强化～强化程度高于周论肝论密度～其中心低密度～三期灶

均论明论强化。提示肝右占位～肝癌可能性大。论论充分论前准论行剖腹叶

探论论～论中可论肝论暗论色～肝论第?段和第?段可论大小论7 cm×6 cm、表面光滑、正常论论分界楚的论论～论中超论告论前相似。切除论物与清声与

包膜完整～论中论病理论告冰:肝血管瘤。常论病理论告,肝血管平滑肌脂肪瘤。HE染色论论,大小不等的论论的血管平滑肌论论;论1 A,～血管周论呈海论皮论胞增生;论状内1 B,。免疫论论化论论,学CD34;+,～HMB-45;-,～SMA;+,～EMA;-,。

2　论论

HAML罕论,系肝论的良性论论性论瘤,多论生于女性。论瘤多论论论～或多性灶生论,1,4,。亦可肝多论瘤性病论同论论生。与内

病理所论其由平滑肌论胞、厚壁血管脂肪论胞论成。论论上主要分论肌论型学

脂肪瘤论型～血管瘤论型和混合型。易平滑肌肉瘤、论性论论论论论胞瘤和与

肝论胞癌混淆。论常论染色～论助免疫论论化学HMB45、CD34、CD31、EMA、SMA等论论,一般均可明论论。确断

HAML彩超多表论论论界楚的论论～回不均～多普勒论论提示论论及论清声匀

论周论可论富的血流信。增强丰号CT多表论论论论密度不均～三期均出论匀

强化。MRI示论瘤呈短T1、论T2信～增强后明论强化～脂肪信亦号号区

出论强化脂肪抑制后短T1论成论T1,1,。本例HAML彩超示论物强回声内声极匀号光论～部回不均～但未论血流信。CT示三期均增强。

有者论论学HAML的论前论主要依影像论论～但论瘤成分的差断靠学异会

论影像的论化,来学8,。CT论论脂肪表论有助于论论论～论物脂肪含量断当

DOC格式论文～方便的论制修改论您减

多论论容易论～而平滑肌成分论多或血管成分论多、脂肪成分论少论论论断断

度论大,1,。肝癌患者多有肝炎病史且论瘤论论不～清CT论论肝硬化病灶中是否有血管影有论论意论。增强CT多表论论论论密度不均～三期均出论匀

强化～藉此可肝论胞癌相论论,与7,。

HAML一旦被确极论～论论手论切除～常论肝部分切除或论瘤及包膜完整切除,6,。本例通论论中超的声运用～使得论中的手论切除范论更加精确内。有论道论瘤包膜外肝论论存在HAML成或向周论肝论论论份展～呈浸论论界～故论论～随确学以定其生物行论,9,。

【参献考文】

[1] 论～论黄曾辰～论论秀～等.肝血管平滑肌脂肪瘤的论断与治论[J].中普国与外基论论床论志～2002～9(6):439-440.

[2] Zhong DR, Ji XL. Hepatic angiomyolipoma-misdiagnosis as hep atocellular carcinoma:A report of 14 cases[J]. World J Gastroenterol, 2000,6(4):608-612.

[3] 李论论～周论平～吴孟超～等.肝血管平滑肌脂肪瘤的论治[J].中论普通外科论志～2004～19(8):504.

[4] Tang LH, Hui P, Garcia-Tsao G, et al. Multiple angiomyolipomta of the liver: a case report[J]. Mod Pathol, 2002,15(2):167-171.

[5] Langner C, Homayounfar K, Ruten B, et al. Concomitant occurre nce of angiomyolipoma, focal nodular hyperplasia , bile duct adenoma,and cavernous hemangioma in the liver[J]. Pathologe, 2001,22(6):417-423.

[6] Dalle I, Sciot R, de Vos R,, et al. Malignant angiomyolipoma of the liver: a hitherto unreported variant[J]. Histopathology, 2000,36(5):443-450.

[7] 任宁～论论秀～论利猷～等.肝血管平滑肌脂肪瘤的论断与治论[J].中论肝胆外科论志～2004～10(6):377-379.

[8] 论～论鑫森祥～肖文波～等.肝血管平滑肌脂肪瘤的影像及学病理分学析[J].浙江大论学学(版医学)～2007～36(1),88-92.

[9] 论论论～论文论～冼志论～等.肝血管平滑肌脂肪瘤的形论论论和学异

免疫论论化学特征[J].中论病理论学志～2004～33(5),437-440.

范文二：肝血管平滑肌脂肪瘤一例

作者:汲武广 陆朝阳 刘连新 田蓝天 王晨宇 姜洪池

【关键词】 血管肌瘤·脂肪瘤·肝肿瘤

1 病例介绍

患者男, 54岁。 于 2008年 3月因体格检查发现肝脏占位来我院住院诊疗, 主诉无明显不适。 入院查体 :皮肤巩膜无黄染,心、肺未见异常,腹平软,肝脾肋下未及,右上腹压痛(-) ,反 跳痛(-) 。辅助检查:甲胎蛋白(alpha fetoprotein, afp) 、癌胚抗原(cacinoembryonic antigen, cea ) 、 ca199 均正常。 肝炎指标及肝功能均正常, b 超示肝右叶可见一大小约 66 mm ×57 mm的略高回声光团,边界清,与肝右静脉关系密切,内部回声极不均匀,可见不规则 低回声, 但未见血流信号, 门静脉主干呈向肝性, 肝内胆管未见扩张。 增强 ct 示肝右叶可见 7 cm ×6 cm 大小低密度肿块,密度不均匀,动脉期病灶边缘见斑片状强化,门脉期及延迟期 病灶边缘明显强化,强化程度高于周围肝脏密度,其中心低密度,三期均见明显强化。提示 肝右叶占位,肝癌可能性大。经过充分术前准备行剖腹探查术,术中可见肝脏暗红色,肝脏 第ⅶ段和第ⅷ段可见大小约 7 cm ×6 cm 、表面光滑、与正常组织分界清楚的肿块,术中超声 报告与术前相似。 切除肿物包膜完整, 术中冰冻病理报告 :肝血管瘤。 常规病理报告:肝血管 平滑肌脂肪瘤。 he 染色检查:大小不等的扩张的血管平滑肌组织(图 1 a ) ;血管周围呈海绵 状内皮细胞增生(图 1 b ) 。免疫组织化学检查:cd34(+) , hmb-45(-) , sma (+) , ema (-) 。 2 讨论

haml罕见 , 系肝脏的良性间质性肿瘤 , 多发生于女性。 肿瘤多为单发, 或多灶性生长 [1,4]。 亦可与肝内多种瘤性病变同时发生。

病理所见其由平滑肌细胞、 厚壁血管脂肪细胞组成。 组织学上主要分为肌样型; 脂肪瘤样型; 血管瘤样型和混合型。 易与平滑肌肉瘤、 恶性纤维组织细胞瘤和肝细胞癌混淆。 经常规染色, 辅助免疫组织化学 hmb45、 cd34、 cd31、 ema 、 sma 等检测 , 一般均可明确诊断。

haml彩超多表现为边界清楚的肿块,回声不均匀,多普勒检查提示团块及团块周边可见丰 富的血流信号。 增强 ct 多表现为肿块密度不均匀, 三期均出现强化。 mri 示肿瘤呈短 t1、 长 t2信号, 增强后明显强化, 脂肪信号区亦出现强化脂肪抑制后短 t1变成长 t1[1]。 本例 haml 彩超示肿物强回声光团,内部回声极不均匀,但未见血流信号。 ct 示三期均增强。

有学者认为 haml 的术前诊断主要依靠影像学检查,但肿瘤成分的差异会带来影像学的变化 [8]。 ct 检查脂肪表现有助于鉴别诊断,当肿物脂肪含量多时较容易诊断;而平滑肌成分较 多或血管成分较多、脂肪成分较少时诊断难度较大[1]。肝癌患者多有肝炎病史且肿瘤边缘 不清, ct 检查肝硬化病灶中是否有血管影有鉴别意义。增强 ct 多表现为肿块密度不均匀, 三期均出现强化,藉此可与肝细胞癌相鉴别[7]。

范文三：肝血管平滑肌脂肪瘤一例

肝血管平滑肌脂肪瘤一例

啦细胞的体外免疫教应功能分析.中国免疫学杂志.1997,13:

131—134

4曹雪涛.章卫平,王建莉,等.白细胞舟索2基因修饰的鼠肝细胞

脾内移植对肝脏免疫功舷的影响.中华医学杂志.1996.76:646—

649.

5C壮唧M,P】诅m-NguyenK,KwongYL.Ad啪删

mediatedinter]eukhn-12genetherapyformeta~tstiecolonear,no-

maprocNatLAcadSeiUSA,1996.93:11302-113o6. 6GuptaS,YemeaiPR.VemuruRP.etalStiesonthemfetyof intr~pLettichepatocytetmnspLamati~:Te1.e'.~rtcetoexvlvogene

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(收藕日期:2000,04—24)

(本文鳊辑沈洁)

肝血管平滑肌脂肪瘤一例

倪型灏陈波

患者男,68岁.1991年体检时B超发现左肝有一直径2

cm的肿块,诊断为"血管瘤",无不适,亦未经任何治疗.

1999年固胸背部带状疱疹复查B超,左肝示7.6cm×7cm

×8.1cm强光团,界尚清,内部略不均匀,印象为左肝血管

瘤.CT检查发现左肝内侧殷有6.9cm×67cm肿块,表面 隆起,其内密度不均(3.3,51Hu),强化至83Hu,动脉期病 灶内可见较粗条状血管影,边缘灶强化,界较清.因CT印象 首先考虑血管平滑肌脂肪瘤.于1999年9月13日住院行左 肝肿块切除术.体检:体温37.c,脉搏80次/min,呼吸20 次/mln,血压21/12kP丑.一般情况好,神清合作,浅表淋巴 结未及肿大.腹平戟,肝剑突下4cm,质戟,肝脾肋下未及. 亦无移动性浊音.实验室检查:肝功能除血清碱性磷酸酶 (116.00U/L).答氨酰转肚酶(72.00U/L)升高外.余均正 常.甲胎蛋白3.50峨/L.CA19-9683U/m1.CEA290

ng/ml.心电图正常.

病理检查:大体观察带部分肝组织的肿块标本一份,总 体积为It)cm×10cm×7?,剖面肿块呈藏红或淡黄色,质 嫩.无包膜,与周围肝组织分界清.瘤体中央区可见退变坏 死.镜下肿瘤由血管,平滑肌和脂肪三种成分组台而成,且 不同区域比例和分布不一,具明显不均一性瘤性肌样细胞 大多呈多边形,圆形或不规则上皮样细胞形态,胞质透亮或 强嗜酸性,嗜酸颗粒可集聚细胞中央呈块状.核大.园,卵圆 或不规则形.居中或塌位.偶见棱内包涵体,形似横纹肌母样 瘤巨细胞.瘤组织多以实性成片或梁索状排列,部分瘤细胞 呈短梭肥胖或长梭形.穿插于上皮样细胞间呈柬状或漩涡交 织状排列,形似分化性平滑肌肉瘤样结构.核分裂象罕见 作者单位:310022杭娴,浙江省肿瘤医院病理科 病例报告?

实性成片或束状肌样细胞问夹杂片状成熟的脂肪细胞或不 规则厚壁血管,偶见小簇状淋巴样细胞授润,示髓外造血改 变.瘤组织可插人周围肝组织中,但界精无移行改变.免疫 组化染色肿瘤Vimetin,HMB45呈阳性;S/vIA,S-100呈灶性 阳性;MG,AFP,cK(AE1/AE3),MAC387,CD68均阴性.病

理诊断:左肝血管平滑肌脂肪瘤.术后患者已脯访11个月, 健在.

讨论:肝血管平滑肌脂肪瘤(HAML)因特定的部位和变 异多样的形态改变,以往绝大多数病例因错诊为原发性肝 癌,血管瘤,平滑肌肉瘤,脂肪内瘤抑或炎性假瘤等而造成误 治.近年来J暄着放射影像学诊断技术的不断进展,术前发现 HAML已成为可能,且报道日益增多,然确切的诊断则仍有 赖于病理组织学的全面观察和免疫组化的证实. HAML大多发生于中老年女性,病理形态学改变类似肾 的血管平滑肌脂肪瘤,通常肿瘤由血管,平滑肌和脂肪组织3 种成分以不同的比例组合而成,其阃尚可有明星的细胞异 型,瘤巨细胞及偶见的蕾外造血现象,通常棱分裂象少见. 鉴于不同病例或同一肿瘤不同区域细胞分化不一.比例不 同,分布形式各异.造成肿瘤细胞形态的多形性,异形性和组 织图像的多样性.为免误诊.诊断者除应熟悉该瘤的组织形 态变异外.应用HMB45和新近发现的色素源性细胞分化标 记物Melan-A(A103)进行免疫组化染色,可证实HAML内肌 样细胞强阳性表达.由此成为能与此类抗体起反应的唯一的 间叶源性肿瘤,具有诊断和鉴别诊断的重要价值.同时,上 述免疫表型的研究结果,也为该瘤组织起源肝血管周围上皮 细胞的观点提供了有力的依据.

(收稿日期:2000—0913)

(本文编辑侯虹鲁)

范文四：肝上皮样血管平滑肌脂肪瘤1例

肝上皮样血管平滑肌脂肪瘤 1例

上皮样血管平滑肌脂肪瘤 (epitheliolid agiomyolipoma, EAML 亦称单形性上 皮样血管平滑肌脂肪瘤 (monotypic ep-itheliolid agiomyolipoma , MEAML) 和不典 型血管平滑肌脂肪癇 (atypical angiomyolipoma, AAML) 。 WHO(2004)泌尿系统和 男性生殖器官肿瘤分类中将 EAML 单独分类,定义为一种具有恶性潜能的间叶 肿瘤。 血管平滑肌脂肪瘤 (agiomyolipoma,AMu主要发生在肾, 肝 AML 是一种较 为少见的肝脏良性间叶性肿瘤, EAML 是 AML 的罕见亚型, 发生在肝脏的 EAML 报道更为罕见。当肿瘤显示不典型的特征时很容易与有些肿瘤混淆。现报道 1例肝 EAML 病例并行文献复习。

1 临床资料

患者男性, 26岁。无诱因上腹不适感 3个月,腹胀,轻微疼痛。本病变发 现于意外交通事故行体检时。 CT 示:肝左叶见 10cm×10cm×8cm 巨大实性占位, 边界清晰, 似有假包膜, 肿块内见较丰富的血流信号, 考虑局灶结节增生可能性 大。胆结石。双侧肾脏及脾未见异常。肝功正常,血清 AFP 、 Cal99阴性。

2 病理检查

巨检:切除肝肿块及胆囊。肝剖面见肿块约 10cm×9cm×7.5cm ,界限较清, 有假包膜,切面灰白间灰红色,实性。质脆。胆囊内见多枚结石。镜检:主要由 多边形或圆形的上皮样肿瘤细胞组成,排列成疏松弥漫的不规则片状或梁索状, 其间见有丰富的畸形厚壁、 薄壁血管, 梭形肌样瘤细胞呈区域性分布, 脂肪成分 不明显。 上皮样瘤细胞呈多边形或圆形类似于肝细胞, 胞质丰富透亮或嗜伊红色, 核圆形或卵圆形,居中或偏位,核仁明显,核分裂象少见,肿瘤间质中可见散在 淋巴细胞浸润。病理诊断:肝透明细胞癌。后经专家会诊及免疫组化证实为: EAML(恶性潜能, 建议, 临床密切随访 ) 。 免疫组化:CK8/18、 CK-L 、 hepatoeyte 、 S-100、 Melan-A 均阴性, HMB~5、 SMA 、 CD34均阳性。

3 讨论

范文五：肝脏血管平滑肌脂肪瘤

肝脏血管平滑肌脂肪瘤的影像学及病理学分析

―――付 10例报道并文献复习

张鑫 严森祥 肖文波 许顺良

作者单位:310003,杭州,浙江大学医学院第一附属医院放射科

摘要 目的 分析肝脏血管平滑肌脂肪瘤的 CT 及 MR 的影像学特点与病理基础, 减少对该 肿瘤的误诊。 材料与方法 回顾性分析 10例 11个病灶肝血管平滑肌脂肪瘤的 CT 及 MR 的 影像学表现,按不同病理组织学亚型进行对照分析。 结果 10例肝脏血管平滑肌脂肪瘤中, 脂瘤型 3例,血管瘤型 1例,肌瘤型 1例,混合型 5例。脂瘤型以脂肪成分为主, CT 、 MR 均可显示脂肪密度或信号。血管瘤型以血管成分为主, CT 、 MR 增强可见动脉期明显强化, 门静脉期密度或信号仍高于同期肝实质。肌瘤型主要是由平滑肌成分为主,脂肪成分很少, 混合型三种成分含量相似, CT 平扫为较均匀低密度, MR 平扫 T1W 呈低信号, T2W 呈稍 高信号,增强扫描 CT 、 MR 均呈动脉期明显强化,门静脉期仍有强化。 结论 不同病理组织 学类型的肝脏血管平滑肌脂肪瘤, CT 和 MR 表现有所不同,这取决于肿瘤所含的主要组织 学成分。 了解肿瘤不同的组织学亚型与影像学表现之间的关系, 有助于提高肝脏血管平滑肌 脂肪瘤术前诊断准确率。

关键词 肝脏 血管平滑肌脂肪瘤 体层摄影术, X 线计算机 磁共振成像

Correlation between pathology and image characteristics of hepatic angiomyolipoma Zhang xin,Yan senxiang,Xiao wenbo,Xu shunliang. Department of Radiology, First Hospital, Medical College of Zhejiang University, Hangzhou, 31003, China

Abstract Objective To discuss the imaging including CT and MRI characteristics of hepatic angiomyolipoma based on pathological findings, with the aim of more accurate diagnosis of the rare lesion of the liver. Material and Method Ten patients with 11 angiomyolipomas were retrospectively analyzed, and their CT and MRI appearances were correlated with the pathohistological subtypes of the disease. Results Ten patients with hepatic angiomyolipomas were subcategorized into lipomatous (3 cases ) , angiomatous(1), myomatous(1) and mixed(5) types, where lesions of the lipomatous type were mainly composed of adipocytes which could be easily recognized on both CT and MRI. Abnormal vessels were commonly seen in the angiomatous lesions, which showed pronounced enhancement in the early arterial phase and remained higher than or isodense with the normal parenchyma in the portal phase. The myomatous type was predominantly composed of leiomyoid cells mixed with small amount of adipocytes. The mixed type was the most often, evenly comprising sheets of epithelioid muscle cells admixed with islands of adipocytes and abnormal vessels, and showing homogeneously low density on plain CT and low signal intensity on T1-weighted, intermediately high signal intensity on T2-weighted MRI scans. On dynamic study with both CT and MRI, the mixed type exhibited obvious enhancement, which retained to some degree during the portal phase. Conclusion In hepatic angiomyolipomas, there showed discrete CT and MRI appearances with different pathological subtypes depending on the components of the tumor. More accurate diagnosis can be achieved with the knowledge of correlation between pathological subtype and imaging characteristics.

Keywords liver angimyolipoma tomography , X-ray computed MRI

肝脏血管平滑肌脂肪瘤(Hepatic Angiomyolipoma HAML)是肝脏少见的良性肿瘤,它 由不同含量的脂肪细胞、平滑肌细胞和畸形血管组成。 HAML 术前诊断困难,易误诊为肝癌、 肝脏血管瘤等。 笔者将 10例经过病理证实的 HAML 病例进行回顾性分析, 旨在提高对本病的 认识。

材料和方法

我院 1999年 2月~2005年 1月经手术病理证实的 HAML 共 10例 11个病灶。 其中男性 3例,女性 7例,男女比例 3:7,年龄 26~64岁,平均年龄 39.9岁。临床症状表现为上腹 部不适伴隐痛 4例,无明显临床症状 6例。 10例患者均无神经系统症状。 10例中 1例有甲 状腺癌手术史, 1例有肾脏血管平滑肌脂肪瘤手术史。实验室检查 HBsAg (-) 9例, HBsAg (+) 1例, 10例肿瘤标记物 AFP 、 CEA 、 CA 125 、 CA 199 均在正常范围。

10例患者均行 CT 平扫及增强扫描。 CT 扫描 4例使用 Toshiba 600 CT 机、 2例使用 Maconi 8000 4排螺旋 CT 机, 4例使用 Toshiba Aquilion 16多排螺旋 CT 机, 扫描矩阵为 512×512, 视野 20cm ×20cm ,层厚 1~10mm ,层间距 0~5mm 。增强扫描使用 60%碘海醇。 10例患者中 5例行了 MR 平扫及增强, MR 检查机型均为 Signa Excite GE 1.5T 超导型核磁共振机。扫描 层厚 8~10mm ,间隔 1~2mm 。常规行横断面 T1WI SE、 T2WI FSE(抑脂)平扫及钆喷葡胺 增强 3期扫描。

结果

按 Tsui 等 [1]提出的根据肿瘤所含的主要组织学成分进行分型的方法对本组 10例 11个病 灶肝脏血管平滑肌脂肪瘤进行病理分型, 其中脂瘤型 3例,血管瘤型 1例, 肌瘤型 1例,混 合型 5例。 肿瘤大小 3.1~14.5cm , 平均 6.7cm 。 术后所有肿瘤大体病理所见肿瘤境界清楚, 均未发现明确包膜。

在 3例脂瘤型肝脏血管平滑肌脂肪瘤中,脂肪含量 >60%。 CT 密度和 MR 信号与皮下脂 肪相似,其中可见条索状、斑片状软组织密度或信号,增强扫描脂肪部分未见强化,软组织 密度或信号部分强化较明显。(图 1)

在 1例血管瘤型肝脏血管平滑肌脂肪瘤中, 病理上以畸形血管为主。 CT 平扫呈低密度, 其内可见点条状钙化, 增强扫描呈动脉明显较均匀强化, 门静脉期密度仍高于同期正常肝实 质。(图 2)

在 1例肌瘤型和 5例混合型肝脏血管平滑肌脂肪瘤中,其主要表现为软组织肿块影, 混合型有 1例肿瘤内可见到灶性脂肪密度或信号, 4例虽然脂肪含量约 30%,但不呈灶性, 为弥漫存在,在 CT 和 MR 上未能显示,增强扫描肿瘤呈明显强化,门静脉期仍有强化, 1例肌瘤型、 2例混合型呈稍低于同期肝实质密度或信号, 2例混合型呈等于同期肝实质密度 或信号, 2例混合型呈稍高于同期肝实质密度或信号。

所有 10例肝脏血管平滑肌脂肪瘤中,有 7例 8个病灶增强扫描肿瘤内或边缘可见粗大 血管显影 (图 4) 。 1例肾脏血管平滑肌脂肪瘤术后患者, 在 CT 、 MR 检查发现同时在肝脏、 肾脏、 后腹膜及后纵隔患有血管平滑肌脂肪瘤。 10例患者术前影像学诊断为 HAML 有 6例, 3例诊断为原发性肝癌, 1例因为有 2年前甲状腺癌手术史,且左右肝内各可见一肿瘤病灶

而诊断为肝脏转移瘤。

讨论

血管平滑肌脂肪瘤 (Angiomyolipoma AML ) 是一种良性间叶性肿瘤, 多发生于肾脏, 发生在肝脏及其他脏器者罕见。 自 Ishak [2]1976年首次报道发生于肝脏的血管平滑肌脂肪瘤, 至今国内外仅有百多例报道, 且多为个例报道, 10例以上的报道较少见并以 CT 影像所见报 道为主, 而笔者搜集了 10例 11个病灶, 并结合病理组织学特点作回顾性分析, 希望在对该 肿瘤的认识和术前诊断准确性上有所助益。

HAML 多见于青年女性, 少数病例可与多发性结节硬化、 肾脏血管平滑肌脂肪瘤并发。 临床上, HAML 常无自觉症状,部分表现为肝区隐痛或右上腹不适。病理上, HAML 是由 不同含量的脂肪细胞、平滑肌细胞和畸形血管组成。 Tsui 等根据 HAML 中的三种成分比例 不同将其分为 4个型:脂瘤型、 血管瘤型、肌瘤型和混合型。 不同病理学类型其影像学表现 亦有所不同。

脂瘤型主要是由分化成熟的脂肪细胞组成,肿瘤周边或其间可见少量畸形厚壁血管和 梭形平滑肌细胞。因而在 CT 平扫示肿瘤组织密度与脂肪相似,并可见条索状、斑片状软组 织密度, MRI 平扫在短 T1长 T2脂肪信号内出现条索状、斑片状稍长 T1稍长 T2信号。增强 扫描脂肪成分无明显强化, 条索状、 斑片状软组织结构强化明显, 这与肿瘤内粗大的畸形血

管有关。肿瘤内存在较多脂肪是脂瘤型 HAML 的特征性表现之一 [3-5]。本型需与脂肪瘤、高分 化型脂肪肉瘤相鉴别。脂肪瘤常由单一脂肪组成, CT 、 MR 呈均匀脂肪密度或信号,增强 扫描无明显强化;肝脏脂肪肉瘤发病率较 HAML 更低,其内软组织成分增强扫描呈轻度强 化 [6],可与动脉期明显强化的 HAML 相鉴别。

血管瘤型是由大量的畸形厚壁血管及平滑肌细胞组成,脂肪成分较少,影像学上往往 见不到。 CT 平扫呈低密度,其内有时可见点条状血管样钙化, MR 呈长 T1稍长 T2信号, 钙化在 MR T1W 、 T2W 均呈低信号,增强扫描肿瘤动脉期明显均质强化,门静脉期仍呈高 于同期肝实质的强化, 但较动脉期有所减退, 延迟期呈低密度或低信号。 术后病理显示肿瘤 内的点条状钙化来源于肿瘤内的厚壁血管。 本型的增强特点需与血管瘤相鉴别, 肝脏血管瘤 常见且常多发, 增强扫描动脉期即出现强化, 但通常在肿瘤边缘出现环形或结节样强化, 随 时间延迟逐渐向肿瘤中心强化, 呈“早出晚归” 的强化特征。仅有较小的血管瘤增强扫描时 呈动脉期均匀强化, 但延迟扫描仍有明显强化。 且 MR 平扫 T2W 为极高信号, 呈 “灯泡征” , 肿瘤内极少见 HAML 所显示的点条状血管样钙化。

肌瘤型主要由平滑肌成分组成, 脂肪成分少于 10%, 混合型病理上表现为实性成片的 肌样细胞混以片状脂肪细胞, 其间穿插着不规则的厚壁血管。 这两型因平滑肌含量较多, 常 呈实质性软组织肿块。 CT 平扫呈稍低密度软组织肿块,其内密度常不均匀, MR 平扫 T1W 呈稍低信号, T2W 呈稍高信号,信号常较混杂,这与肿瘤内成分混杂有关。增强扫描肿瘤 动脉期强化较明显, 门静脉期仍有强化, 呈稍高或等于同期肝实质密度、信号,延迟扫描肿 瘤边缘可出现假包膜样环形强化。 本型需与肝细胞性肝癌、 肝腺瘤等相鉴别。 肝细胞性肝癌 动态增强扫描呈也呈 “快进快出”的特点,且延迟期扫描肿瘤边缘可见假包膜强化。病理学 显示 HAML 没有假包膜形成,影像学所见假包膜样强化在术后与病理切片对照研究发现主 要是由肿瘤周围的炎性细胞浸润和扩张的小胆管而形成。 此现象文献报道很少 [7], 且在 T2W 此假包膜样改变呈高信号,而肝细胞癌的假包膜在 T2W 呈低信号,此点可与肝细胞癌相鉴 别。本组病例行 MR 检查较少,此现象还有待进一步验证。且肝细胞肝癌常有肝炎、肝硬 化病史,血 AFP 检查常增高。肝腺瘤在肿瘤周边可见脂肪密度、信号环,增强扫描肿瘤动 脉期明显强化, 但中心瘢痕无强化, 门静脉期及延迟期强化减退呈低密度、 信号, 中心疤痕 强化明显,周边环无强化。

有人报道典型 HAML 的肿瘤血管呈卷发状或旋涡状,但出现率低 [8]。笔者复习文献并 结合本组病例发现,无论是哪种病理类型 HAML ,因其内含有畸形厚壁血管,且常较粗大, 所以 MR 平扫肿瘤内有时可见流空的血管影像,增强扫描常可见强化的粗大血管。本组 3例增强扫描且行肝动脉血管重建可见肝动脉分支延伸至肿瘤内,这在其他肿瘤少见,是 HAML 的又一特征性影像表现 [9-10]。

综上所述,不同病理类型的肝脏血管平滑肌脂肪瘤因其所含的组织学成分比例不同, 可呈不同的影像学改变, 了解它们之间的关系把握特征性的影像学表现, 有助于我们术前做 出准确诊断。

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World J Gastroenterol. 2003 Aug;9(8):1856-8. Related Articles,

Links

Diagnosis and treatment of hepatic angiomyolipoma in 26 cases.

Ren N, Qin LX, Tang ZY, Wu ZQ, Fan J.

Liver Cancer Institute, Fudan University, Shanghai 200032, China. ningren@zshospital.com

AIM: To summarize the experience of the diagnosis and treatment of hepatic angiomyolipoma (HAML). METHODS: The clinical, imaging and pathological features, and treatment strategies of 26 patients with HAML treated at the authors' institute between October 1998 and January 2003 were retrospectively analyzed. All the patients received liver resection and were followed up till the study. Immunohistochemical assays were performed with a panel of antibodies. RESULTS: There was an obvious female predominance (21:5), and most of the patients (18/26) had no symptoms. Heterogeneous high echo was found in ultrasonography and punctiform or filiform vascular distribution pattern was found in color Doppler-sonography in most of the lesions (21/26). All of the 5 lesions further enhanced with Levovist showed early and prolonged enhancement. At contrast-enhanced spiral CT, the soft-tissue components of 24 lesions were markedly enhanced in the arterial

phase and 18 lesions remained enhanced in the portal venous phase. MRI was performed in 9 patients, and showed hypointensity or hyperintensity on T1-weighted images and heterogeneous

hyperintensity on T2-weighted images. Histopathologically, all lesions were composed of adipose tissues, smooth muscle and blood vessels with different proportions. Most lesions showed positive immunohistochemical staining for HMB45 (26/26), A103 (24/26) and SMA (24/26). All of the 26 patients showed a benign course with no sign of recurrence. CONCLUSION: Preoperative radiological

diagnosis of HAML is possible. The demonstration of intratumoral fat and central vessels is helpful in the diagnosis. HMB45, A103 and SMA are promising markers for pathologic diagnosis of HAML, and surgical resection is effective for the treatment of HAML.

Angiomyolipoma of the liver with least amount of fat component: imaging features of CT, MR, and angiography.

Yoshimura H, Murakami T, Kim T, Nakamura H, Hirabuki N, Sakon M, Wakasa K, Inoue Y.

Department of Diagnostic Medicine, Course of Biosystem Medicine, Osaka University Graduate School of Medicine D1, 2-2, Yamadaoka, Suita City, Osaka 565-0871, Japan.

We report two cases of angiomyolipoma of the liver, where small amounts of fat on computed tomography, magnetic resonance imaging, and angiography made distinguishing this tumor from other hypervascular tumors difficult. Recognizing the imaging features of no capsule, hypervascularity of the tumor including the fat component, and early venous drainage may be helpful for correctly diagnosing angiomyolipoma of the liver.

Eur Radiol. 2001;11(8):1389-95. Related Articles,

Links

Correlation of MRI and CT findings with histopathology in hepatic angiomyolipoma.

Hogemann D, Flemming P, Kreipe H, Galanski M.

Department of Radiology, Hannover Medical School, Germany. hoegeman@helix.mgh.harvard.edu

Hepatic angiomyolipomas are rare and often mimic other liver tumors. The aim of our study was to describe the CT and MRI findings and to correlate imaging features with histopathology. The CT and/or MR images were available for retrospective analysis in seven

patients. Patients had non-enhanced as well as enhanced CT (n = 6) or MRI (n = 4) before and after administration of Gd-DTPA (n = 2) or MnDPDP, a liver specific contrast agent, (n = 3). In three patients CT and MRI did not detect fat, and in two patients the angiomyolipomas were also histopathologically devoid of fat. Vascularity ranged from hypervascular (n = 4) with arteriovenous shunts (n = 1) to equal (n = 1) or less (n = 2) postcontrast enhancement compared with the normal liver parenchyma. No uptake of the liver specific contrast agent, MnDPDP, was observed (n = 3). Predominantly, CT and MRI did not include angiomyolipoma in the differential diagnosis, and the initial histopathological evaluation was inconclusive in more than half the cases. Hepatic angiomyolipomas frequently manifest as solitary

well-circumscribed heterogeneous masses in patients with no underlying liver disease or elevation of serum tumor markers. If present, the demonstration of intratumoral fat is helpful in the diagnosis of angiomyolipoma. The final diagnosis can be obtained by immunohistochemistry

Abdom Imaging. 1998 Sep-Oct;23(5):520-6. Related Articles,

Links

Angiomyolipoma of the liver: significance of CT and MR dynamic study.

Ahmadi T, Itai Y, Takahashi M, Onaya H, Kobayashi T, Tanaka YO, Matsuzaki Y, Tanaka N, Okada Y.

Department of Radiology, Institute of Clinical Medicine, University of Tsukuba, Ibaraki, Japan.

Angiomyolipoma is a benign mesenchymal tumor that has been reported frequently in the kidney but rarely in the liver. In the present study, we present four cases of hepatic angiomyolipoma with different radiologic appearances, discuss differential diagnosis, and review previously reported cases. One of our cases was followed for 8 years. Computed tomography (plain, enhanced, and dynamic study), magnetic resonance imaging (T1-weighted spin echo, T2-weighted spin echo, and dynamic study), ultrasonography, and angiography were performed in all cases. Although different radiologic appearances were observed in the tumors, based on different proportions of fat, blood vessels, and muscle, three cases were diagnosed as angiomyolipoma. In one case, it was quite

difficult to make radiologic diagnosis; hepatocellular carcinoma with fatty metamorphosis in part was most likely suspected, but histopathological examination revealed angiomyolipoma with few fat elements. In the present study, early and prolonged enhancement of the lesion with the special pattern of time density/intensity curve was significant for angiomyolipoma, and we suggest that preoperative radiologic diagnosis of the lesion is possible in most of the cases. However, it can be quite difficult to distinguish angiomyolipoma from some hepatocellular carcinomas with fatty metamorphosis.

Am J Surg Pathol. 1999 Jan;23(1):34-48. Related Articles,

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Hepatic angiomyolipoma: a clinicopathologic study of 30 cases and delineation of unusual morphologic variants.

Tsui WM, Colombari R, Portmann BC, Bonetti F, Thung SN, Ferrell LD, Nakanuma Y, Snover DC, Bioulac-Sage P, Dhillon AP.

Department of Pathology, Caritas Medical Centre, Kowloon, Hong Kong.

Hepatic angiomyolipoma (AML) is frequently misdiagnosed. HMB-45 is a promising immunomarker for this tumor that leads to recognition of some AMLs with unusual morphology. The purpose of this collaborative study is to better define the morphologic variations of AML. Thirty AMLs were examined, including four biopsy specimens and two fine-needle aspirates. The diagnosis was confirmed by the presence of HMB-45-positive myoid cells. Almost half the cases were originally misdiagnosed as carcinomas or sarcomas. There was marked female predominance (25:5), and the mean age was 48.7 years (range 29-68). Three patients (10%) had evidence of tuberous sclerosis and all had renal AML. According to the line of differentiation and predominance of tissue components, the tumors was subcategorized into mixed, lipomatous (> or = 70% fat), myomatous (< or="10%" fat),="" and="" angiomatous="" type.="" the="" mixed="" type="" was="" the="" most="" common="" (11="" resected="" cases),="" comprising="" sheets="" of="" epithelioid="" muscle="" cells="" admixed="" with="" islands="" of="" adipocytes,="" abnormal="" vessels,="" and="" frequently,="" hematopoietic="" cells.="" six="" tumors="" (including="" three="" from="" biopsy="" specimens)="" were="" heavily="" fatty="" and="" showed="" predominantly="" adipocytes="" with="" epithelioid="" and="" short="" spindle="" myoid="" cells="" webbed="" between="" fat="" cells.="" of="" 10="" myomatous="" amls,="" five="" tumors="" showed="" a="" pure="" sinusoidal="" trabecular="" pattern="" and="" comprised="" mainly="" epithelioid="" cells.="" typically,="" mature="" adipocytes="" were="" absent="" or="" scanty,="" but="">

was seen as fine droplets within cytoplasm or as occasional large globules in sinusoids. Pelioid and inflammatory pseudotumor-like patterns were identified focally. Regarding cellular features of the myoid cells, most of the epithelioid cells were either eosinophilic or clear with spiderweb cell morphology. Three AMLs showed an almost purely oncocytic appearance with scanty fat. Large pleomorphic epithelioid cells existed as small foci. Spindle cells arranged in long fascicles were uncommon. D-PAS-positive globules were common around pelioid areas. Brown pigments with staining characteristics of hemosiderin and/or melanin were noted. In conclusion, we propose HMB-45-positive myoid cells as the defining criterion of hepatic AML, which is a tumor capable of dual myomatous and lipomatous differentiation and melanogenesis. Because of its protean morphologic appearance, recognition of the various variant patterns and cell types is important for a correct diagnosis, assisted by immunohistochemical confirmation with HMB-45. Trabecular and oncocytic cell tumors appear to stand out as distinctive subtypes.

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