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Lingnan Modern Clinics in Surgery ›› 2020, Vol. 20 ›› Issue (01): 34-37.DOI: 10.3969/j.issn.1009-976X.2020.01.008

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Clinical analysis of hepatic perivascular epithelioid cell neoplasm: A report of two cases

  

  1. Department of Hepatobiliary Surgry, The First Affiliated Hospital of Jinan University, Guangzhou, Guangdong 510630, China
  • Contact: CAO Ming-Rong

肝脏血管周上皮样细胞瘤2例临床诊治分析

  

  1. 暨南大学附属第一医院肝胆外科,广州 510630
  • 通讯作者: 曹明溶

Abstract: [Abstract] Objective To investigate the clinical features of hepatic perivascular epithelioid cell carcinoma (PEComa) and the experiments in the diagnosis and treatment to hepatic PEComa. Methods The clinical data of two patients with hepatic PEComa at The First Affiliated Hospital of Jinan University from August 2018 to November 2019 were collected. The clinical manifestations, imaging features, diagnostic and therapeutic strategies, pathologic features, prognosis were analyzed. Results The two patients with hepatic PEComa were female, with no specific clinical symptoms and signs. The tumor indicators such as AFP, CEA and ca-199 were negative, and the imaging diagnosis was liver cancer. Two patients received a surgical resection and no recurrence or metastasis was observed. Conclusion Hepatic PEcoma is a rare mesenchymal tumor, which is mainly benign and difficult to diagnose before surgery. Histological and immunochemical examination are the main methods of diagnosis. The main treatment measures are surgical resection and long-term close follow-up.

Key words: liver tumour, perivascular epithelioid cell neoplasms (PEComa), diagnose, therapeutics

摘要:

肝脏血管周上皮样细胞瘤2例临床诊治分析

刘康寿, 曹明溶*

[摘要] 目的 探索肝脏血管周上皮样细胞瘤(PEComa)的疾病特点和诊治方案。方法 收集暨南大学第一附属医院2018年8月至2019年11月2例肝脏血管周上皮样细胞瘤患者的临床资料,分析其临床表现、影像学特点、诊断及治疗策略、病理特点、预后。结果 2例肝脏PEComa患者均为女性,没有典型的临床症状及体征,AFP、CEA、CA-199等肿瘤指标均为阴性,B超、上腹MRI等影像学检查结果考虑为肝癌,治疗上采取腹腔镜肝肿物切除术,术后复查未见肿瘤复发及远处转移征象。结论 肝脏PEcoma是少见的间叶源性肿瘤,以良性为主,术前诊断误诊率高,组织学及免疫组化学检查是确诊的主要方法。主要治疗措施是手术切除及长期密切随访。

[关键词] 肝脏肿瘤;血管周上皮样细胞瘤(PEComa);诊断;治疗

血管周上皮样细胞肿瘤(PEComa)是一类在组织学和免疫组化上有独特表型的间叶源性肿瘤。常说的PEComa是指PEComa-NOS,即PEComa中的非特殊类型。其可以发生于女性生殖系统、消化系统、泌尿系统和软组织等部位,但原发的肝脏PEComa十分罕见[1]。此类型的PEComa大部分为良性肿瘤。另外,还有一种特殊类型的PECo-ma,其中包括:肾与肾外血管平滑肌脂肪瘤(AML)、淋巴管肌瘤病(LAM)、肺透明细胞糖瘤(CCST)及透明细胞肌黑色细胞肿瘤(CCMMT)。此类型的PEComa恶性可能性较高[2]。PEComa肿瘤家族的定义时间短,发病率低,可在全身多个脏器发病,误诊率高,治疗及预后尚不明确。本次报道收集暨南大学第一附属医院2018年8月至2019年11月2例肝脏血管周上皮样细胞瘤患者的临床资料,分析其临床表现、影像学特点、诊断及治疗策略、病理特点、预后,以提高对该疾病的诊治经验。

1 病案摘要

病例1:患者女性,30岁,因“腹胀半年,检查发现肝右叶肿块16天”入院。患者半年前出现腹胀,多在夜间饮水后出现,无畏寒、发热、恶心、呕吐、腹痛、腹泻、便秘、消瘦、皮肤及巩膜黄染等不适。16天前在外院检查行全腹MRI示:肝右叶肿块,肝细胞癌可能性大。为求诊治,拟“肝右叶占位性质待查”收入我科。既往体健,无特殊。体格检查:皮肤、巩膜无黄染,浅表淋巴结无肿大,心肺正常,腹部平软,无压痛,肝肋缘下未触及,肝区无叩击痛,肝浊音界存在,余无特殊。

入院后完善三大常规、肝肾功能、凝血功能、AFP、CA-125、CA-153、CA-199、CEA、乙肝两对半、乙肝病毒DNA定量、丙肝抗体及胃肠镜检查,均未见明显异常。肝脏B超结果示:肝右前叶一低回声团,性质待定。全腹MRI平扫+增强示:肝S8段占位,考虑肝细胞癌可能性大。于2018-08行腹腔镜肝脏肿物切除术,术中见肝表面光滑,无肝硬化结节,VII段可见一肿物突出肝脏表面,大小约4 cm×4 cm×3 cm,质硬。见图1、表1。

图1 病例1的肝脏影像学图像、手术标本及免疫组化染色

A:病例1肝脏B超图像;B:病例1肝脏MRI T1图像;C:病例1肝脏MRI T2图像;D:病例1手术切除标本;E、F:病例1免疫组化染色

表12 例肝脏PEComa的MRI表现

时期、序列动脉期门脉期延迟期T1 T2 DWI病例1不均匀强化强化程度迅速减退强化程度迅速减退低信号不均质稍高信号稍高信号病例2不均匀强化强化迅速减退强化迅速减退稍低信号混杂高信号高信号

术后病理结果肉眼观察:肝组织一块:8 cm×7 cm×4.5 cm,已切开,切面见一直径6 cm的肿物,呈多彩状,边界清。镜检:镜下见肿瘤细胞胞质丰富,透亮或淡嗜伊红色,呈圆形或多边形,细胞核圆形或椭圆形,泡状核,核仁明显,核偏位,核分裂小于1个/5CHPF,瘤细胞呈滤泡状排列,瘤细胞巢之间为丰富、纤细的血管网,肿瘤伴有出血,局部泡沫状细胞聚集。免疫组化:AFP(-)、Hepatocyte(-)、Glypican-3(-)、CD10(-)、CD34(-)、CK8(-)、Syn(-)、CgA(-)、CD68(+)、CD163(-)、Mac387(-)、CD1a(-)、S-100(-)、TFE-3(-)、SMA(-)、Des-min(-)、Vim(+)、MelanA(+)、HMB-45(少数,+)、HBCAg(-)、HBSAg(-)、P-CK(-)、ki-67约5%(+)、PAS染色(-)、网状纤维染色(+),符合肝脏PECo-ma-NOS。见图1、表2。

病例2:患者女,70岁,因“体检发现肝内占位性病变15天”入院,患者15天前在我院体检行肝胆彩超发现肝内低及稍强回声结节,性质待定。随后行上腹MRI平扫+增强提示:肝S3、S4占位,考虑肝Ca可能性大。查AFP、CA-199、CEA未见异常。自觉无畏寒、发热、恶心、呕吐、腹痛、腹泻、便秘、消瘦、皮肤及巩膜黄染等不适。为求诊治,拟“肝占位性质待查:肝癌?”收入我科。既往体健,无特殊。体格检查:皮肤、巩膜无黄染,浅表淋巴结无肿大,心肺正常,腹部平软,无压痛,肝肋缘下未触及,肝区无叩击痛,肝浊音界存在,余无特殊。见图2。

入院后完善三大常规、肝肾功能、凝血功能、CA-125、CA-153、乙肝两对半、乙肝病毒DNA 定量、丙肝抗体及胃肠镜检查,均未见明显异常。于2019-09-02行腹腔镜肝左叶肿物切除术,术中见肝脏表面光滑,质软,未见肝硬化结节,左肝外叶近肝圆韧带处可见一大小约5 cm×4 cm肿物,边界欠清,质软。

术后病理结果肉眼观察:肝组织一块,大小3.7 cm×2.0 cm×0.8 cm,切面灰黄色病变区,质中,与正常肝组织分界清。镜检:镜下检肿瘤由血管周上皮样细胞,脂肪细胞和厚壁血管混合组成,细胞呈圆形,中央细胞质嗜伊红色,周边胞质呈透亮状,核圆形或椭圆形。免疫组化:HMB-45(+)、β-catenin(+)、Melan-A(+)、SMA(+)、CD34/CD31(血管,+)、Desmin(-)、ki-67 约 1%。符合血管周上皮样细胞分化的肿瘤(PEComa)。见图2、表2。

图2 病例2的肝脏影像学图像、手术标本及免疫组化染色

A:病例2肝脏B超图像;B:病例2肝脏MRI T1图像;C:病例2肝脏MRI T2图像;D:病例2手术切除标本;E、F:病例2免疫组化染色

表2 2例肝脏PEComa的免疫组化染色结果

指标HMB-45 Melan-A SMA Desmin CD34 Ki-67病例1病例2++---5%+++-+1%

2 讨论

1992年Bontti等人把构成血管平滑肌脂肪瘤(AML)、肺透明细胞瘤(CCST)、淋巴管肌瘤(LAM)的组成细胞定义为血管周上皮样细胞(PEC)[3]。这一类细胞通常分布于血管周围,围绕血管腔呈放射状排列,其胞质丰富、含透明嗜酸性颗粒,通常对HMB-45、Melan-A、SMA、Desmin等免疫组化标记呈阳性。其他免疫组化标记如s-100,CD34和CDll7也有阳性可能,但表达情况并不总是一致[4]。2002年世卫将PEComa定义为由和血管壁呈特殊关系,能够特异性表达黑色素和肌细胞标志物的细胞组成的间叶源性肿瘤[5,6]。

肝脏PEComa极为罕见,目前尚未见相关的大宗病例报道。本病多发生于女性,在任何年龄段均可出现,临床表现常无特异性,多数病人无自觉症状或偶有出现右上腹不适感,仅少数病人会出现消瘦、贫血等恶病质症状。血液检查方面,AFP、CEA、CA-199等肿瘤指标在肝脏PEComa病人上多无特异性表现,且暂未发现其发病与肝炎病毒感染有关[7,8]。CT、MRI、B超等影像资料对于发现肝脏PEComa具有重要作用,但由于缺乏特征性的影像学表现,导致其鉴别诊断难度大,误诊率高。Zhao等分析并总结了22例肝脏PEComa的影像学资料,结果显示:在CT表现中,该肿瘤多表现为形状规则的界清软组织肿块。肿块密度较均匀,内部含脂肪组织、囊性变或囊内出血时可表现出混杂密度。如患者存在有肝硬化或脂肪肝,病灶可表现为等密度或者高密度。在MRI表现中,T1WI多显示为低信号,T2WI多显示为高信号,DWI多显示为高信号。在CT和MRI的增强扫描中,动脉期病灶不均匀强化,门静脉期持续强化,但强化程度较前下降,延迟期则表现出强化明显减退。由于增强扫描后多出现类似于肝癌的“快进快出”现象,故常误诊为肝癌[9]。目前肝脏PEcoma多需组织病理学和免疫组化学确诊。在组织病理学上,常见PEC细胞分布于血管周围,围绕血管腔呈放射状排列,其胞质丰富、含透明嗜酸性颗粒,偶尔可见凝固型坏死和出血。在免疫组化学上,黑色素生成标志物(HMB-45、Melan-A)阳性和平滑肌肌动蛋白SMA阳性为其特征性表现,有时可以表达肌源性标志物如弹性蛋白抗体(Vim)及血管源性标志物CD34等[10]。本次报道的2例患者中,HMB-45、Melan-A均为阳性,1人SMA阳性。

绝大部分的肝脏PEComa是良性的,恶性者极为罕见。对于肝脏PEComa良性与恶性的鉴别。良性PEComa不应具备以下任一条件,而恶性PEComa应具备以下2项或以上条件[11]:①肿瘤最大直径大于5 cm;②浸润性生长;③高级别核级;④细胞丰富;⑤核分裂象>1个/50 HPF;⑥肿瘤内部出现凝固性坏死;⑦侵犯周围血管。肝脏PECo-ma对放化疗的敏感性较差,手术治疗并达到R0切除是首选的治疗方案[12]。如今,腔镜技术和设备快速地发展,腹腔镜下肝肿物切除可成为此类病人手术切除的首选方式。由于PEComa与肝癌的术前鉴别较为困难,所以手术应保证足够的切缘,争取达到肿瘤的R0切除。此外,病人术后的定期随访极为重要,术后第一年每隔3个月复查一次肿瘤指标及肝脏影像学检查,第二年起可每半年复查一次。本次报道的2例病人均接受了腹腔镜肝肿物切除术,均达到R0切除标准,效果满意。术后分别随访时间分别为15个月和3个月,期间复查肿瘤指标、肝胆彩超、肝脏MRI等均未见肿瘤复发与转移征象。

参考文献

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Clinical analysis of hepatic perivascular epithelioid cell neoplasm:A report of two cases

LIU Kang-Shou,CAO Ming-Rong
Department of Hepatobiliary Surgry,The First Affiliated Hospital of Jinan University,Guangzhou,Guangdong 510630,China

[Abstract] Objective To investigate the clinical features of hepatic perivascular epithelioid cell carcinoma(PEComa)and the experiments in the diagnosis and treatment to hepatic PEComa.Methods The clinical data of two patients with hepatic PEComa at The First Affiliated Hospital of Jinan University from August 2018 to November 2019 were collected.The clinical manifestations,imaging features,diagnostic and therapeutic strategies,pathologic features,prognosis were analyzed.Results The two patients with hepatic PEComa were female,with no specific clinical symptoms and signs.The tumor indicators such as AFP,CEA and ca-199 were negative,and the imaging diagnosis was liver cancer.Two patients received a surgical resection and no recurrence or metastasis was observed.Conclusion Hepatic PEcoma is a rare mesenchymal tumor,which is mainly benign and difficult to diagnose before surgery.Histological and immunochemical examination are the main methods of diagnosis.The main treatment measures are surgical resection and long-term close follow-up.

[Key words] liver tumour;perivascular epithelioid cell neoplasms(PEComa);diagnose;therapeutics

doi:10.3969/j.issn.1009-976X.2020.01.008

中图分类号:R735.3

文献标识码:A

作者单位:暨南大学附属第一医院肝胆外科,广州510630

*通讯作者:曹明溶:教授,主任医师,博士生导师,Email:tcaomr@jnu.edu.cn

Corresponding author:CAO Ming-Rong,tcaomr@jnu.edu.cn

(收稿日期:2019-12-17)

关键词: 血管周上皮样细胞瘤(PEComa), 肝脏肿瘤, 诊断, 治疗

CLC Number: