子宫血管周上皮样细胞肿瘤临床病理学观察

子宫血管周上皮样细胞肿瘤(perivascular epithelioid cell tumor,PEComa)是一种非常罕见的肿瘤,极易误诊.作者在病理诊断中诊断1例,结合文献复习报道如下.     

子宫血管周上皮样细胞肿瘤3例临床病理分析

目的探讨子宫血管周上皮样细胞肿瘤(perivascular epithelioid cell tumor, PEComa)的临床病理特征、诊断及鉴别诊断等。方法采用免疫组化EnVision两步法对3例子宫PEComa进行检测,并复习相关文献。结果 3例肿瘤由梭形细胞和上皮样细胞构成,胞质透明至嗜酸性,间质血管丰富,其中1例肿瘤细胞异型性显著,并见出血、坏死。免疫表型:3例HMB-45阳性,2例SMA、Caldesmon阳性,Melan-A、TFE-3、desmin、CD10、CD117和S-100蛋白均阴性,Ki-67增殖指数5%～30%。随访4～55个月,患者均存活。结论子宫PEComa是一种少见的间叶源性肿瘤,结合组织学形态及免疫表型可辅助诊断。     

子宫血管周上皮样细胞肿瘤1例

患者,68岁,以"阴道不规则出血3 个月"为主诉,月经规律4～5 天/30天,56 岁绝经.3 个月前阴道出血,量少;3天前阴道出血增多,因症状持续存在而就诊.彩超示:子宫后位,长径38 mm,前后径28 mm,横径35 mm,内膜厚3 mm,轮廓清晰,形态正常,肌层回声不均.子宫宫底部可见大小21 mm ×15 mm...     

子宫血管周上皮样细胞肿瘤2例报道及文献复习

子宫血管周上皮样细胞肿瘤（perivascular epithelioid cell tumor,PEComa）是一种少见的肿瘤,有其特有的镜下特点和免疫组化特征。现收集2例子宫PEComa病例,并结合文献探讨其诊断和鉴别诊断,以提高对该病的认识。     

子宫血管周上皮样细胞肿瘤临床病理观察

目的研究子宫血管周上皮样细胞肿瘤的病理学特征、诊断、鉴别诊断和生物学行为。方法对5例子宫血管周上皮样细胞肿瘤进行常规组织学和免疫组织化学（SP法）染色和观察,对患者进行随访,并复习相关文献。结果光镜下5例肿瘤均由透明或嗜酸细胞巢或宽窄不等的细胞索组成,间质有丰富的小血管和程度不等的透明变。免疫组织化学染色示5例瘤细胞均黑色素细胞标记阳性和程度不等的结蛋白和平滑肌肌动蛋白（SMA）阳性,CK和CD10阴性。5例患者现均存活。结论子宫血管周上皮样细胞肿瘤具有较特征性的组织病理及免疫组织化学特点,HMB45阳性对诊断有重要作用。该肿瘤分良性、恶性潜能不能确定和恶性三类,应与透明细胞癌和上皮样平滑肌肿瘤区别。     

鼻腔血管周上皮样细胞肿瘤临床病理分析

目的 探讨血管周上皮样细胞分化肿瘤(neoplasm with perivascular epithelioid cell differentiation,PEComa)的临床病理特征,并对国内外相关文献进行复习,以提高对PEComa的认识和病理诊断水平.方法 对1例PEComa进行临床表现分析,对其病理组织学和免疫组化染色进行观察,并结合国内外相关文献复习PEComa的特点.结果 患者发现左鼻腔无痛渐大性肿物伴反复出血1年余.组织病理学形态表现为裂隙状血管周围见片巢状排列的上皮样细胞,细胞胞质透亮,内含淡红色细颗粒状,核小且一致,圆形或卵圆形,核仁小,核分裂罕见.免疫表型:HMB-45、Melan-A、vimentin和SMA(+);S-100蛋白、CK和EMA(-).结论 发生于鼻腔的PEComa罕见,诊断主要依靠病理学,手术切除为首选治疗,其为低度恶性潜能肿瘤,需长期随访.     

血管周上皮样细胞肿瘤的分子遗传学

血管周上皮样细胞肿瘤(perivascular epithelioid cell tumours,PEComas)是由组织学和免疫组织化学上有独特表现的血管周上皮样细胞构成的间叶性肿瘤,PEComas家族包括肾血管平滑肌脂肪瘤、肺透明细胞"糖"瘤、淋巴管肌瘤病、淋巴管平滑肌瘤、镰状韧带透明细胞肌黑色素细胞性肿瘤和其他部...     

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(perivasculaRepithelioid 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PEComas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4.5～10 cm,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/10 HPF.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.     

子宫外上皮样滋养细胞肿瘤

患者女，28岁。1998年行产钳分娩一男婴，同年早孕人流一次。2001年12月4日，因停经48天，阴道流血10d，近4天出现下腹隐痛伴肛门附胀及里急后重来院就诊，拟诊“宫外孕”收治入院。     

恶性血管周上皮样细胞肿瘤2例并文献复习

目的 探讨恶性血管周上皮样细胞肿瘤(malignant perivascular epitheliod cell tumor,PEComa)的临床、病理特征及鉴别诊断.方法 对2例恶性PEComa进行临床、病理形态学、免疫表型及电镜观察,并复习相关文献.结果 镜下见肿瘤组织由梭形细胞及上皮样细胞构成,细胞异型性明显,细胞胞质透明或嗜酸,细胞核大,有核仁,瘤细胞由纤细的纤维结缔组织及薄壁血管将其分隔成巢状、腺泡状、片状结构,部分区域可见多核瘤巨细胞伴肿瘤坏死,核分裂多见.免疫表型:肿瘤细胞表达HMB-45、S-100、SMA及desmin;电镜下见肿瘤细胞胞质内可见高电子密度核心的内分泌小体和少许不成熟的黑色素小体.结论 恶性PEComa罕见,病理形态多样,熟悉其临床、病理形态、免疫表型及电镜下改变,有助于临床、病理医师对其正确诊断及鉴别诊断.     

Perivascular epithelioid cell tumor (PEComa) in the genitourinary tract

Perivascular epithelioid cell tumors (PEComas) are mesenchymal tumors composed of histologically, immunohistochemically, ultrastructurally, and genetically distinctive cells. PEComas have been described in different organs and are considered ubiquitous tumors. In this review we discuss recent informations related to PEComas in the genitourinary tract.     

Perivascular epithelioid cell sarcoma (malignant PEComa) of the ileum

Epithelioid angiomyolipoma (AML) is the prototype of a heterogeneous group of lesions characterized by the presence of HMB-45 positive cells with clear cytoplasm, perivascular distribution, and combined myomelanocytic features, so-called perivascular epithelioid cells (PECs). These lesions are being increasingly referred to as PEComas. PEComas have been reported at diverse anatomic sites, but mainly in the abdominopelvic cavity and rarely in parenchymatous organs, skin, and soft tissues. Gastrointestinal (GI) PEComas are exceptionally rare, with less than 10 cases documented so far. Rare examples of PEComas with pleomorphic histology could have been misinterpreted as unusual variants of carcinoma or sarcoma. To make a contribution to the differential diagnosis of difficult-to-classify pleomorphic GI sarcomas, we report on a malignant pleomorphic neoplasm with features of PEComa involving the terminal ileum in a 63-year-old woman. Fourteen months after resection of the primary tumor, a huge abdominopelvic recurrence was successfully resected, but no distant metastases were detected. The differential diagnosis and malignancy criteria of GI PEComas will be discussed.     

Perivascular epithelioid cell tumour (PEComa) of the soft tissue

AIMS: PEComa is a rare tumour developing from perivascular epithelioid cells (PEC) and is characterised by positive immunoreactivity for HMB45. Since PEComas are tumours having both a spindle cell component and an epithelioid and giant cell component, as seen in many sarcomas, as well as having a wide distribution in various organs and soft tissue, we reviewed cases originally diagnosed as sarcomas of the soft tissue in our institution and screened them by immunostaining for HMB45. METHODS: Consecutive soft tissue sarcomas (31 tumours) retrieved from the Surgical Pathology file at our institution for a period of 3 years were submitted for immunostaining for HMB45. Cases with positive HMB45 immunostaining were submitted for further immunostaining for MART1, CD68, S100 protein, cytokeratin AE1/3, EMA, vimentin, MSA and CD117. RESULTS: Of 31 sarcomas, three tumours in the group of 11 malignant fibrous histiocytomas (MFH) and unclassified sarcomas showed positive immunoreactivity for HMB45 and MART1 in 1-25% of tumour cells. The three tumours were located in the lower extremities and measured 8, 11 and 12 cm in diameter. Patient gender male:female was 2:1 and ages were 46, 56 and 60 years. Microscopically, the tumours were composed of a variable proportion of spindled cells, multinucleated cells and epithelioid cells disposed in diffuse sheets or nests. Mitotic figures and necrosis were frequent. The immunoreactivity was diffuse for CD68, focal for AE3 and EMA, negative or focal for MSA and CD117, and negative for S100 and AE1. All three patients developed lymph node or distant metastases and died of the disease within 1-2 years. CONCLUSIONS: PEComa re-screened from the group of high grade sarcomas without definite differentiation range from pleomorphic to monomorphic cytohistopathological features. Immunostaining for HMB45 of unclassified sarcomas is useful for the classification of these tumours. They occur preferentially in the lower extremities and have a high malignant potential when associated with large size, tumoural necrosis and high mitotic activity.     

Perivascular epithelioid cell tumor (PEComa) of soft tissue: case report with ultrastructural study

A perivascular epithelioid cell tumor (PEComa) arising in the abdominal wall of a 44-year-old female is described. The lesion was a well-circumscribed but unencapsulated, rubbery, subcutaneous mass measuring 3.5 x 3.5 x 3.0 cm which was removed by simple excision. It was characterized by a nest- or sheet-like arrangement of round to polygonal cells with round nuclei and abundant clear to slightly eosinophilic cytoplasm containing glycogen. There was mild to moderate nuclear pleomorphism with mitotic activity of 6 per 10 high power fields. A short fascicular proliferation of the tumor cells was observed focally. The stroma contained abundant small vascular channels with hyalinization. Immunohistochemically, the tumor cells were strongly positive for vimentin, epithelial membrane antigen, alpha-smooth muscle actin, and HMB45. Ultrastructural examination showed poorly differentiated mesenchymal tumor cells without premelanosomes. There was local recurrence 6 years after excision. Pathologists and clinicians should be aware of the existence of PEComa in soft tissue and should differentiate it from other similar lesions.     

肠道血管周上皮样细胞肿瘤三例临床病理学特征分析

目的:探讨肠道血管周上皮样细胞肿瘤（PEComa）的临床病理学特征。方法:收集3例肠道PEComa病例的资料,光镜下观察HE、免疫组织化学和特殊染色切片,应用荧光原位杂交（FISH）法检测TFE3基因断裂重组情况,复习相关文献并进行分析总结。结果:肿瘤主要位于肠道黏膜下层和肌层,界限较清,细胞呈上皮样或胖梭形,2例排列...     

Pancreatic perivascular epithelioid cell tumor (PEComa)

Neoplasms with perivascular épithelioid-cell differentiation (PEComas) are rare tumors with a distinctive immunoreactivity for melanocytic markers. They have been described in various organs. We report an intrapancreatic PEComa discovered in a 46-year-old woman during a workup for diarrhea. CT scan showed a 1.7cm nodule in the body of the pancreas with slight-contrast enhancement at arterial time and isodense at portal time. The aspect was suggestive of an endocrine tumor despite negative somatostatin-receptor scintigraphy. Enucleation was performed. Pathologic evaluation showed a well-circumscribed intrapancreatic tumor consisting of a population of clear to eosinophilic spindle cells and a less abundant population of epithelioid cells arranged around blood vessels. Tumor cells expressed vimentin, HMB45 and actin and only focally S-100 protein, KL1, CD117 and CD34. These features were consistent with a PEComa. Pancreatic PEComas are rare, but should be included in the differential diagnostic of pancreatic clear cells tumors or pancreatic spindle- and epithelioid-cells tumors.     

Perivascular epithelioid cell tumor (PEComa) of the pancreas: Immunoelectron microscopy and review of the literature

A perivascular epithelioid tumor (PEComa) is a rare tumor probably arising from the perivascular epithelioid cells. Only three cases of pancreatic PEComa have been reported in the English-language literature. The present report describes an extremely rare case of pancreatic PEComa. A 47-year-old Japanese woman complained of lower abdominal pain and a well-demarcated solid tumor was found in the pancreatic head. There was no history of tuberous sclerosis complexes. Pylorus-preserving pancreaticoduodenectomy was thus performed. There was a well-demarcated, solid tumor measuring 17 mm in the pancreatic head. The tumor was composed of a diffuse proliferation of epithelioid tumor cells with many blood vessels but no adipose tissue. The tumor cells expressed HMB45 and α-smooth muscle actin. Ultrastructurally, the tumor cells possessed many membrane-bound granules that were positive for HMB45 on immunoelectron microscopy. The results of immunoelectron microscopy show that some PEComas possess not only typical melanosomes or premelanosomes but also aberrant melanosomes.     

Perivascular epithelioid cell tumor (PEComa) of the liver: a case report and review of the literature

Perivascular epithelioid cell tumor (PEComa) is rare entity and has been described only recently. By immunohistochemistry and genetics it belongs to the family of tumours which comprises angiomyolipoma, clear cell "sugar" tumor of lung, lymphangioleiomyomatosis and clear cell myomelanotic tumor of ligamentum falciforme/teres hepatis. We describe an unusual case of hepatic PEComa arising in a 55-year-old woman with previous history of glioblastoma. Histologically the tumor grew in expansive way, and was composed of clear and eosinophilic epithelioid cels, without vascular or lipomatous component characteristic of angiomyolipoma. There was mild nuclear pleomorphism, sporadic mitotic activity and haemorrhage without necrosis. On immunohistochemistry, the tumor was HMB-45+50, Melan-A and smooth muscle actin positive. Tyrosinase, S-100 protein, cytokeratin coctail, EMA, vimentin, muscle specific actin, CD10, TTF-1, hepatocyte, desmin and cyclin D1 were negative. Sporadic nuclear p53 positivity was seen. The main differential diagnosis of hepatic PEComa includes clear cell variant of liver cell adenoma and hepatocellular carcinoma, metastases of various clear cell carcinomas and metastasis of malignant melanoma. In respect of uncertain biologic potential of PEComa, long term follow up is indicated.     

A huge malignant perivascular epithelioid cell tumor (PEComa) of the uterine cervix and vagina

Perivascular epithelioid cell tumors (PEComas) are a family of rare mesenchymal neoplasms, including angiomyolipoma, clear-cell “sugar” tumor of the lung and extrapulmonary sites, lymphangioleiomyomatosis, clear-cell myomelanocytic tumor of the falciform ligament/ligamentum teres, and clear-cell tumors at various other anatomic sites.