Hepatic perivascular epithelioid cell tumor:Clinicopathological analysis of 26 cases with emphasis on disease management and prognosis

BACKGROUND Perivascular epithelioid cell tumor(PEComa)is an uncommon tumor of mesenchymal origin.Cases of PEComa in the liver are extremely rare.AIM To analyze the clinicopathological features and treatment of hepatic PEComa and to evaluate the prognosis after different treatments.METHODS Clinical and pathological data of 26 patients with hepatic PEComa were collected.All cases were analyzed by immunohistochemistry and clinical follow-up.RESULTS This study included 17 females and 9 males,with a median age of 50 years.Lesions were located in the left hepatic lobe in 13 cases,in the right lobe in 11,and in the caudate lobe in 2.The median tumor diameter was 6.5 cm.Light microscopy revealed that the tumor cells were mainly composed of epithelioid cells.The cytoplasm contained heterogeneous eosinophilic granules.There were thick-walled blood vessels,around which tumor cells were radially arranged.Immunohistochemical analysis of pigment-derived and myogenic markers in PEComas revealed that 25 cases were HMB45(+),23 were Melan-A(+),and 22 SMA(+).TFE3 and Desmin were negative in all cases.All the fluorescence in situ hybridization samples were negative for TFE3 gene break-apart probe.Tumor tissues were collected by extended hepatic lobe resection or simple hepatic tumor resection as the main treatments.Median follow-up was 62.5 mo.None of the patients had metastasis or recurrence,and there were no deaths due to the disease.CONCLUSION Hepatic PEComa highly expresses melanin and smooth muscle markers,and generally exhibits an inert biological behavior.The prognosis after extended hepatic lobe resection and simple hepatic tumor resection is semblable.

Perivascular epithelioid cell neoplasm of the colon

A 17-year-old female presented with rectal bleeding from an ulcerated sigmoid mass in 1994.Initial pathological evaluation revealed a rare clear cell neoplasm of the colon,possibly originating from kidneys,adrenals,lung or a gynecologic source as a metastatic lesion.Extensive imaging studies were negative,and over the next 15 years,she remained well with no recurrence.The original resected neoplasm was reviewed and reclassified as a perivascular epithelioid cell neoplasm (PEComa).Although the long-term natural history of PEComas requires definition,increased clinical and pathological awareness should lead to increased recognition of an apparently rare type of colonic neoplasm that likely occurs more often than is currently appreciated.

Perivascular epithelioid cell tumors of the liver misdiagnosed as hepatocellular carcinoma:Three case reports

BACKGROUND Hepatic perivascular epithelioid cell neoplasms(PEComas)are rare.Diagnostic and treatment experience with hepatic PEComa remains insufficient.CASE SUMMARY Three hepatic PEComa cases are reported in this paper:One case of primary malignant hepatic PEComa,one case of benign hepatic PEComa,and one case of hepatic PEComa with an ovarian mature cystic teratoma.During preoperative imaging and pathological assessment of intraoperative frozen samples,patients were diagnosed with hepatocellular carcinoma(HCC),while postoperative pathology and immunohistochemistry subsequently revealed hepatic PEComa.Patients with hepatic PEComa which is misdiagnosed as HCC often require a wider surgical resection.It is easy to mistake them for distant metastases of hepatic PEComa and misdiagnosed as HCC,especially when it’s combined with tumors in other organs.Three patients eventually underwent partial hepatectomy.After 1-4 years of follow-up,none of the patients experienced recurrence or metastases.CONCLUSION A clear preoperative diagnosis of hepatic PEComa can reduce the scope of resection and prevent unnecessary injuries during surgery.

Retroperitoneal neoplasm with perivascular epithelioid cell differentiation: A case report and review of literature

The retroperitoneal neoplasm with perivascular epithelioid cell differentiation (PEComa) is an extremely rare pathological entity. In this article, we reported one case of a 45-year-old woman who was admitted to our hospital (The Second People's Hospital of Hefei, China) for retroperitoneal neoplasm with perivascular epithelioid cell differentiation. The B ultrasonic examination showed echopoor in the region of cavitas pelvis. The histologic characteristics and immunohistochemical phenotype both revealed the neoplasm with perivascular epithelioid cell differentiation.

肝脏血管周上皮样细胞肿瘤(Pecoma)临床病理分析并文献复习

目的:探讨肝脏血管周上皮样细胞肿瘤的临床病理表现及鉴别诊断。方法:回顾其科室诊断的1例肝脏Pecoma的HE染色表现及免疫组化表达,并复习相关文献。结果:血管周上皮样细胞肿瘤(Pecoma)来源于间叶,组织学表现为肿瘤细胞以上皮样细胞为主,胞浆可透明或嗜酸性,亦可有梭形细胞,间质富于薄壁血管,脂肪细胞少见,免疫组化恒定表达HMB45。结论:肝脏Pecoma相较发生于肾、肺部位少见,具有特殊的组织学表现及特征的免疫标记表达。生物学行为不确定,手术完整切除为主要治疗手段。

Feasibility of laparoscopic isolated caudate lobe resection for rare hepatic mesenchymal neoplasms

BACKGROUND Mesenchymal tumors such as perivascular epithelioid cell neoplasm(PEComa)and inflammatory pseudotumor-like follicular dendritic cell sarcoma(IPT-like FDC sarcoma)are relatively uncommon in the liver and are particularly rare in the caudate lobe.The clinical manifestations and available imaging tests lack specificity for hepatic mesenchymal tumors.To the best of our knowledge,no caudate PEComa or IPT-like FDC sarcoma has been completely resected by laparoscopy.The standard laparoscopic technique,surgical approaches,and tumor margins for potentially malignant or malignant caudate mesenchymal tumors are still being explored.AIM To assess both the safety and feasibility of laparoscopic resection for rare caudate mesenchymal neoplasms.METHODS Eleven patients who underwent isolated caudate lobe resection from 2003 to 2017 were identified from a prospective database.Three consecutive patients with rare caudate mesenchymal tumors underwent laparoscopic resection.Patient demographic data,intraoperative parameters,and postoperative outcomes were assessed and compared with the open surgery group.RESULTS All procedures for the three resection patients with caudate mesenchymal tumors were completed using a total laparoscopic technique by two different approaches.The average operative time was 226 min,and the estimated blood loss was 133 mL.The average length of postoperative hospital stay was 6.3±0.3 d for the laparoscopy group and 15.5±2.3 d for the open surgery group(P<0.05).There were no perioperative complications or patient deaths in this series.CONCLUSION Laparoscopic isolated caudate lobe resection for rare mesenchymal neoplasms is a feasible and curative surgical option in selected patients.

宫颈血管周上皮样细胞肿瘤一例

血管周上皮样细胞肿瘤(perivascular epithelioid cell tumor,PEComa)是一组临床上少见的间叶源性肿瘤,主要发生在围绝经期和绝经后的女性,原发肿瘤多位于子宫和腹膜后等部位,其中子宫PEComa多发生于子宫体浆膜下,少数患者发生于宫颈及黏膜下,恶性者罕见。报告1例因考虑腹盆腔占位及多发骨转移就诊的宫颈PEComa,该患者因左膝关节反复疼痛在当地医院行骶髂关节CT,发现腹盆腔占位性病变及双侧髂骨及腰骶尾骨多发高密度灶,2023年7月收治后结合影像学检查和盆腔肿物穿刺活检考虑子宫肌瘤恶变,排除手术禁忌证后,在全身麻醉下行腹式全子宫切除术+双侧附件切除术+盆腔淋巴结活检+盆腔粘连松解术。术后病理提示宫颈PEComa、淋巴管平滑肌瘤病,出院后定期门诊复诊,术后5个月未见复发。宫颈PEComa的临床表现、影像学表现缺乏特异性,临床上易误诊、漏诊,病理诊断是金标准,目前主要治疗手段为手术切除肿瘤组织。

Single-center Experience in the Diagnosis and Treatment of Hepatic Perivascular Epithelioid Cell Neoplasm

Background and Aims:Perivascular epithelioid cell neo-plasms(PEComas)are a rare type of mesenchymal neo-plasm and their preoperative diagnosis is challenging.In this study,we summarized the experience from a single medical center to study the examinations,clinical presen-tations,and pathological and histological characteristics of PEComas in the liver in order to optimize overall un-derstanding of the diagnosis and treatment of these neo-plasms.Methods:We conducted a retrospective analysis to investigate the clinical and pathological characteristics as well as imaging presentations of 75 patients diagnosed with hepatic PEComa in The First Affiliated Hospital of Zhe-jiang University between April 2010 and April 2020.Re-sults:Among the 75 patients,52 were women,and the median age was 48 years.Most patients had no specific symptoms,and two were admitted to the hospital for a second time owing to relapse.All patients underwent surgi-cal resection.Histologically,38 patients had classical angio-myolipoma(AML)and 37 had epithelioid AML.The PECo-mas were accompanied by positive immunohistochemical expression of HMB45,Melan-A,and smooth muscle actin.Follow-up data were obtained from 47 of the total 75 pa-tients,through October 2020.Two patients had metastasis after surgery.Conclusions:AML is the most common type of hepatic PEComa.There are no specific symptoms of he-patic PEComa,and serological examinations and imaging modalities for accurate preoperative diagnosis are lacking.Epithelioid AML should be considered a tumor of uncertain malignant potential;however,the prognosis of PEComa af-ter resection is promising.

肝脏血管周上皮样肿瘤7例报告并文献复习

目的：探讨肝脏血管周上皮样肿瘤的临床病理学特征、诊断及鉴别诊断.方法：复习山西省肿瘤医院自2008年3月到2015年3月诊治的肝脏7例标本,观察其组织病理学形态及免疫组化结果,并复习相关文献.结果：7例患者平均发病年龄51.5岁（范围41-56岁）,男性2例,女性5例.其中经典型2例,肌细胞为主型3例,脂肪细胞为主型2例.免疫组织化学染色显示：7例病例HMB45均为阳性,6例阳性SMA及VIM,5例阳性Melan A,2例结蛋白阳性,Ki-67平均阳性指数为1.3%（范围0-5%）, TFE3均为阴性.结论：原发性肝脏血管上皮样肿瘤是一种肝脏罕见肿瘤,应提高对其认识和诊疗水平,术后应长期密切随访.免疫组织化学染色标记HMB 45、Melan A和SMA对于肝脏PEComa的诊断具有帮助作用。

肝脏血管周上皮细胞肿瘤与肝细胞肝癌及肝血管瘤的超声特征分析

目的:提高彩色多普勒超声对于肝脏血管周上皮细胞肿瘤的诊断水平及与肝细胞肝癌和血管瘤的鉴别。方法:搜集肝脏血管周上皮细胞肿瘤、肝细胞肝癌、肝血管瘤患者,回顾性分析这些患者的一般情况、超声及超声造影特征,并总结分析。结果:在一般资料中,三组在性别、年龄、症状、AFP水平、HBsAg指标中有统计学差异,P<0.05,在常规超声中,三组在大小、边界、高回声边缘、主要回声、内部低回声、点状或短线状强回声、回声分布、后方回声、血流分布、病灶数量、肝脏背景指标中有统计学差异,P<0.05,在超声造影中,三组在动脉期增强强度无统计学差异,大部分都表现为高增强,P>0.05,但是在增强方式上是有区别,三组在开始增强时间、达峰时间、开始消退时间有统计学差异,P<0.05。结论:肝脏血管周上皮细胞肿瘤在临床特征、超声及超声造影有一定的特点,在与肝细胞肝癌及肝血管瘤鉴别时,常规超声联合超声造影能提高该病的诊断准确率。

4例胃肠道血管周上皮样细胞肿瘤临床病理学分析

目的 探讨胃肠道血管周上皮样细胞肿瘤（perivascular epithelioid cell tumor，PEComa）的临床及病理学特点。方法回顾性复习4例胃肠道原发血管周上皮样细胞肿瘤的病理切片及临床资料，选取典型蜡块做相关的免疫组化染色，抗体包括黑色素相关抗原HMB45、melan—A、肌源性标记抗原SMA、desmin，以及vimentin、CgA、CK、S-100、CD117、CD34。结果4例PECo—mas中男性3例，女性1例，年龄分别为36、38、42及45岁。其中2例位于升结肠，1例位于降结肠，1例位于乙状结肠。肿瘤大小4．5～10cm，境界清楚，切面灰白色，质地均匀，局部可见出血。镜检：肿瘤细胞呈上皮样排列，细胞质丰富，透亮或嗜酸性颗粒状，细胞核空泡状，有明显的核仁，间质富于毛细血管、血窦以及厚壁血管。细胞异型性小，个别病例局部可见轻-中度异型性，分裂象0～3个／10HPF。免疫组化结果：肿瘤弥漫表达HMB45（4／4），弥漫或片状表达vimentin（4／4）、SMA（4／4）以及desmin（3／4）。CgA、Syn、CK、S-100、CD117、CD10及CD34均阴性。4例患者行局部肠管及肿瘤切除，术后随访8、15、32及36个月均无复发和肿瘤转移。结论胃肠道PEComa少见，为低度恶性潜能肿瘤，形态类似于软组织和其他部位的同类肿瘤，手术切除为首选治疗。

子宫恶性血管周上皮样细胞肿瘤一例报道并文献复习

子宫恶性血管周上皮样细胞肿瘤(PEComa)是一种罕见的妇科肿瘤,术前易误诊。本文通过回顾性分析1例诊断为子宫颈恶性PEComa患者的临床资料、影像学表现及病理特点,结合目前子宫恶性PEComa的研究进展进行文献复习,以提高广大医务工作者对该疾病的认识。

胰腺血管周上皮样细胞肿瘤的临床病理特点

目的:探讨胰腺血管周上皮样细胞肿瘤(perivascular epithelioid cell tumors,PEComas)的临床病理学特征、诊断、鉴别诊断、治疗方法和预后.方法:对1例罕见的胰腺PEComas进行光镜观察、免疫组织化学染色,并结合文献探讨该病的临床病理学特点.结果:肿瘤位于胰头部,光镜下可见肿瘤内含有丰富的厚壁和薄壁血管,血管周围及血管间为成巢、成片或器官样排列的上皮样细胞.肿瘤细胞体积较大,呈多边形,胞质丰富,透亮或颗粒状、淡嗜酸性.细胞核小,圆形、卵圆形或不规则形,核分裂象罕见.免疫组织化学显示肿瘤细胞HMB45、Melan-A、SMA、HHF35、Vimentin弥漫强阳性,D2-40和S-100呈弱阳性表达,Syn、CK(Pan)、CD117、CD34、Insuin、Somatostatin、Gastrin、Cal-citonin、α-hCG、ACTH、Serotonin、PP、VIP、Glucagon均阴性.结论:胰腺PEComas是一种生物学行为未定的间叶源性肿瘤,有其独特的组织形态及免疫组织化学表型.目前尚无明确的良、恶性诊断标准.以手术完整切除为主要治疗方法,术后密切随访.

肝恶性血管周上皮样细胞肿瘤临床病理观察

目的研究肝恶性血管周上皮样细胞肿瘤的临床病理学特点、诊断与鉴别诊断。方法收集1例肝恶性血管周上皮样细胞肿瘤的临床病理资料,并复习相关文献,总结其临床病理特点及免疫组化情况。结果本例组织形态学表现为肿瘤间质富于毛细血管、血窦。肿瘤细胞以上皮样细胞为主,大部分细胞的胞质嗜伊红色,小部分细胞透亮,部分细胞质内可见褐色色素颗粒。细胞异型显著,核分裂象易见,并可见病理性核分裂及凝固性坏死。PAS染色(+)。免疫组化:肿瘤细胞HMB45和melanoma(pan)弥漫强(+),部分细胞vimentin和SMA(+),Ki 67增殖指数70%。结论肝恶性血管周上皮样细胞肿瘤是一种少见的肿瘤,诊断时应注意与其他肿瘤相鉴别,尤其是肝细胞癌、恶性黑色素瘤以及转移性肾透明细胞癌。

子宫血管周上皮样细胞肿瘤1例报道并文献复习

目的:探讨妇科血管周上皮样细胞肿瘤(perivascular epithelioid cell tumor,PEComa)的临床表现,病理及免疫表型特点,诊断与鉴别诊断,治疗及预后。方法:讨论我院于2016年收治的一例66岁女性患者,该患者以阴道大量出血入院,初步诊断为子宫肌瘤,行全子宫加双侧附件切除术,术后病理回报为子宫PEComa,针对该病例资料报道并进行相关文献复习。结果:该例子宫PEComa发生于肌壁间,伴广泛出血坏死,细胞有异型性,HMB45(部分+),Melan A(+)、SMA(-)。结论:发生于子宫的血管周上皮样细胞肿瘤是一种少见的间叶性肿瘤,子宫PEComa生物学性质仍不明确,已有资料报道恶性病例,治疗以手术完整切除为主,放化疗疗效仍不明确。长期随访是关键。

子宫血管周上皮样细胞肿瘤研究进展

子宫血管周上皮样细胞肿瘤是一种临床罕见的间叶性肿瘤,其生物学行为尚不确定,恶性者比较罕见。此类疾病发病率低,病因不明确,临床及影像学表现不典型,以中老年女性多见,常因体检发现腹部包块而就诊,通常免疫表型可出现特征性双重表达:同时表达黑色素特异性抗体45(HMB45)和肌源性标志物平滑肌肌动蛋白(SMA)。新近研究发现转录因子E3(translocation facor E3,TFE3)与疾病的发生有一定相关性。Folpe按危险程度将其分为良性、恶性潜能未定型及恶性。该病术前诊断困难,容易与子宫上皮样平滑肌瘤、低级别子宫内膜间质肉瘤、恶性黑色素瘤及透明细胞肉瘤相混淆,目前国内外尚无规范化诊治方案,在实际临床工作中,手术治疗为主要的治疗手段,肿瘤完整切除是有效的治疗方法。放化疗效果不确切,术后应坚持长期随访。

肝脏恶性血管周上皮样细胞瘤临床病理观察

目的探讨肝脏血管周上皮样细胞瘤的临床病理特点和诊断标准。方法对1例恶性病例的临床、影像学和病理特征进行观察并复习文献。结果患者因肿瘤复发死亡。肿物切面灰红灰黄色,质软易碎;镜下见梭形-上皮样瘤细胞围绕薄壁血管放射状排列,核异型深染,可见核分裂象;免疫组化示瘤细胞Vimentin、SMA和MelanA阳性表达,HMB45和CD117阴性表达,Ki-67指数10%。结论肝脏恶性血管周上皮样细胞瘤异型显著,可见核分裂象,CD117阴性表达,Ki-67指数高。

子宫血管周上皮样细胞肿瘤一例报告及文献复习

血管周上皮样细胞肿瘤(PEComa)是一种临床少见的间叶性肿瘤,生物学特性多样,恶性潜能未确定,发生于子宫极为罕见,术前极易漏诊、误诊,临床医生必须给予足够重视。为提高对此病的认识,现对1例子宫PEComa患者资料进行回顾分析,并复习相关文献,进一步探讨其临床表现、病理特点、免疫表型、治疗及预后,以加深临床医生对此病的认识,并提高诊治能力。

子宫血管周上皮样细胞肿瘤临床病理分析

目的探讨子宫血管周上皮样细胞肿瘤（PEComa）的临床病理及免疫组化特征、鉴别诊断及预后。方法对3例子宫PEComa进行临床、光镜及免疫组化的研究并复习相关文献。结果 3例子宫PEComa发病年龄从33至43岁,1例剖宫产偶然发现,2例表现为阴道异常出血,肿瘤大小从1.5cm×1.2cm×0.8cm至4cm×3.5cm×3cm。镜检：肿瘤内广泛分布分支状毛细血管网至透明变性的厚壁大血管,上皮样瘤细胞围绕血管周围呈放射状排列,未见核分裂象及坏死灶,其中1例可见脉管浸润。免疫组化：HMB-45、Melan-A、SMA、Actin及Desmin胞浆阳性。3例均手术切除,随访5-8年不等均无复发或转移。结论子宫PEComa是具有独特的病理学特征的罕见肿瘤,免疫组化黑色素和肌源性标记物双向表达阳性有助于诊断,良性肿瘤手术切除预后好。

肾恶性血管周上皮样细胞肿瘤的临床病理特征

目的:探讨肾恶性血管周上皮样细胞肿瘤(PEComa)的临床病理特征、免疫表型及分子遗传学特点、诊断与鉴别诊断以及治疗方式。方法:通过深入学习2例肾恶性血管周上皮样细胞肿瘤的组织形态特点并复习相关文献,归纳该肿瘤的诊断和鉴别诊断要点、探讨其免疫表型和分子遗传学特征,总结现有的治疗策略。结果:2例分别发生在48岁女性及62岁男性患者,肿瘤直径均大于5 cm,均以上皮样细胞为主,体积大,胞浆丰富,嗜酸性,单核或多核,细胞异型性明显,可见大片坏死,核分裂象约(2~3)个/50 HPF。在例1中,可见丰富纤薄的间质血管将肿瘤分隔成巢状,腺泡样结构;在例2中,肿瘤囊性变,呈多囊性结构,囊壁由肿瘤细胞构成,上皮样细胞间可见梭形细胞区域。2例肿瘤HMB45均局灶阳性,MelanA弥漫强阳性,TFE3蛋白表达均阴性。在例1中,肿瘤细胞不表达肌源性标记(SMA、Desmin均阴性);而在例2中,肿瘤的梭形细胞区域SMA和Desmin局灶阳性。结论:肾恶性血管周上皮样细胞肿瘤属罕见疾病,需对该肿瘤加强学习认识,避免漏诊和误诊,治疗主要以手术切除为主,术后多予随访,但预后较差,分子靶向的个性化治疗将成为趋势。