伴上皮性囊肿的血管平滑肌脂肪瘤1例并文献复习

目的 探讨伴上皮性囊肿的血管平滑肌脂肪瘤(angio-myolipoma with epithelial cysts,AMLEC)的临床病理学特征、诊断、鉴别诊断及预后.方法 回顾性分析1例AMLEC的临床病理学特征、免疫表型等,并复习相关文献.结果 患者女性,49岁,体检发现双肾囊肿2年,CT示右肾囊性肿块.眼观:肿...     

上皮样型肾血管平滑肌脂肪瘤2例

例1,男性,25岁,因左侧腰部疼痛1年余入院。查体:左肾区隆起,可触及8 cm×8 cm×4 cm肿块,质硬,无压痛。B超探及左肾9·2 cm×10·1 cm不均匀包块,提示左肾占位病变。CT考虑左肾癌。手术见左肾14 cm×10 cm×10 cm,肿物将肾皮质推向外侧,肿物表面血管曲张,部分呈囊性感,与毗邻分界清,周围淋巴结未见肿大,行左肾切除。眼观:已剖开肾脏,15 cm×10 cm×6 cm。肿物位于实质,10 cm×5 cm×5 cm,中心大部分出血坏死,呈暗红色血块状,易破碎,与肾组织分界清,部分有包膜。镜检:瘤细胞体积大,圆形或多边形,胞质丰富红染并融合,边界不清,部分胞质淡染…     

肝血管平滑肌脂肪瘤2例并文献复习

目的探讨肝血管平滑肌脂肪瘤(hepatic angiomyoli-poma,HAML)的临床病理学特征、诊断与鉴别诊断、治疗及预后。方法对2例HAML进行临床病理学和免疫组化分析,并复习相关文献。结果 2例HAML均为女性,肿瘤均位于肝左叶。镜下示肿瘤均由血管、上皮样或梭形平滑肌细胞及脂肪3种成分混合组成。2例均见髓外造血。免疫表型:HMB-45、Melan-A和SMA均阳性。结论 HAML好发于女性,是一种少见的良性肿瘤,免疫组化检测有助于诊断与鉴别诊断。     

肾脏不典型血管平滑肌脂肪瘤4例报道并文献复习

目的：探讨肾脏不典型血管平滑肌脂肪瘤（ａｔｙｐｉｃａｌ ａｎｇｉｏｍｙｏｌｉｐｏｍａ,ＡＡＭＬ）的临床病理特征。方法：收集４例曾误诊为肾肉瘤样癌或恶纤组的ＡＡＭＬ行光镜、免疫组化观察,并对其中１例和另３例典型ＡＭＬ作电镜观察。结果：ＡＡＭＬ具有以下的病理特点：①肉眼上切面呈棕褐色,可伴有坏死及出血;②光镜下表现为富细胞,主要由胞浆丰富嗜酸性的单核或多核多形性瘤巨细胞和梭形的肉瘤样细胞及胞浆透明成片     

肾血管平滑肌脂肪瘤31例临床病理分析

目的：探讨肾血管平滑肌脂肪瘤（angiomyolipoma，AML）的临床病理特征。方法：对31例肾AML的临床病理特征进行分析，并做免疫组化检测，其中4例做电镜观察。结果：31例肾AML按其组织形态可分为典型型（67.7%）、非典型型(6.5%)、平滑肌瘤样型（9.7%）、脂肪瘤样型（9.7%）、炎症型（6.5%）5型。免疫表型：肿瘤细胞特征性表达HMB45、Melan-A、gp-100、HHF35、SMA、CD68。其中1例电镜下肿细胞内可见黑色素小体。结论：肾AML的组织形态多样，特征性免疫级化标记为诊断提供依据。掌握形态特征及其鉴别诊断要点有助于临床的正确治疗。     

上皮样血管平滑肌脂肪瘤的临床病理特征

目的:探讨上皮样血管平滑肌脂肪瘤(epithelioid angiomyolipoma,EAML)的临床病理学特征.方法:回顾性分析17例病理诊断为EAML的组织学形态、免疫组织化学特点、临床及随访资料.结果:男5例,女12例,年龄21~62(平均31.2)岁,其中14例发生于肾脏(3例伴有子宫平滑肌瘤,2例伴有宫内孕);2例发生于肝脏,1例发生于腹腔;肿瘤最大径3~15 cm,镜下瘤细胞呈上皮样巢、片状分布,可见围绕血管呈放射状排列的结构;细胞体积大,卵圆形、梭形,胞质丰富,异型明显,核分裂少见,其中2例伴有出血、坏死.免疫组织化学HMB-45,Melan-A,SMA阳性,vimentin,ER和PR表达率分别为41%(7/17),23%(4/17)以及35%(6/17).17例EAML均行肿瘤根治或肿瘤切除术,术后随访5~52个月,1例2年后肝肺转移死亡,其余16例均健在,无复发及转移.结论:EAML多发生于女性,可能与激素水平有关;肾脏最常见,其次为肝、腹腔;病理形态多样,免疫组织化学对其诊断有重要价值,手术效果好,有转移可能.     

恶性上皮样肾血管平滑肌脂肪瘤一例

患者女,28岁。因头晕心慌在心内科就诊时发现腹部肿块,经B超检查发现肾脏巨大肿瘤伴瘤内出血,于2005年1月16日人院。体检：患者自6岁开始有多次癫痫发作,青春期后发作逐渐减少,无家族史。人院后检查可见面部血管纤维瘤（该体征过去曾经被描述为皮脂腺瘤）,腹部可触及肿块,未见其他异常体征。CT示左肾区巨大不规则混杂密度团块影,[第一段]     

右肾巨大血管平滑肌脂肪瘤破裂一例

右肾巨大血管平滑肌脂肪瘤破裂一例王玉珍左东升王淑强患者男，４５岁。因午饭后１小时突然腹部剧痛、呕吐伴腹部膨胀１０分钟，于１９９６年４月５日下午１小时５０分钟急诊入院。入院后查体：患者急性病容，表情淡漠，神志模糊，面色苍白，四肢湿冷，腹部膨胀如足月妊娠...     

儿童肾脏上皮样血管平滑肌脂肪瘤病理特点

目的 探讨儿童肾脏上皮样血管平滑肌脂肪瘤(epithelioid angiomyolipoma,EAML)免疫标记的表达特点及其对临床病理诊断、鉴别诊断和预后的意义.方法 对1例肾脏EAML临床资料进行复习,进行光镜观察和免疫组化染色,并随访.结果 光镜下肿瘤由典型性的上皮样细胞组成,细胞核有异型性,核分裂象可见,有坏死和出血;淋巴结内未见上皮样肿瘤细胞累及.免疫标记:瘤细胞vimentin、SMA、HMB-45、Melan-A、CD68均阳性;而CK(AE1/AE3)、EMA、S-100、CD10、CD34、CD117、ER、PR、Ki-67和p53均为阴性.患者手术后状态良好,无肿瘤局部复发和转移征象.结论 肿瘤以上皮样细胞增生特征为主,临床表现和组织学表现易与肾脏肿瘤及其他肿瘤相混,寻找经典血管平滑肌脂肪瘤的结构和肿瘤细胞表达HMB-45和SMA对诊断和鉴别诊断至关重要.而细胞的不典型性、核分裂、出血和坏死可能只表明肿瘤的恶性潜能;淋巴结受累、肾静脉瘤栓均不是恶性的诊断依据;远处转移才是恶性的证据.Ki-67和p53对患者的预后可能有指示意义.     

多部位发生的肾血管平滑肌脂肪瘤二例

例1男,16岁。以无诱因持续性腹部胀痛伴恶心3 d,于2001年11月3日入院。体检:一般情况尚可,腹部平坦,腹肌紧张,压痛,无反跳痛。于左、右侧上腹部均扪及肿物,边界不清,压痛,肾区无叩击痛。彩色超声检查:双侧肾脏体积明显增大,右肾下极可见一13 cm×10 cm略强回声光团,     

肝脏单形性上皮样血管平滑肌脂肪瘤

目的：探讨肝脏单形性上皮样血管平滑肌脂肪瘤的临床病理学特征及诊断、鉴别诊断要点。方法：对1例单形性上皮样血管平滑肌脂肪瘤进行临床病理学分析及免疫组织化学研究。结果：肝脏单形性上皮样血管平滑肌脂肪瘤临床多无症状,光镜下单形性上皮样血管平滑肌脂肪瘤由形态多样的上皮样细胞构成,胞质透明或嗜酸,无脂肪组织及异常血管;免疫表型;HMB45阳性,SMA及vimentin部分阳性,desmin少数阳性,S－100蛋白弱阳性,cytokeratin及AFP阴性,CD34血管内皮细胞阳性。结论：肝脏单形性上皮样血管平滑肌脂肪瘤是极为罕见的间叶性肿瘤,组织起源至今不明,其诊断及鉴别诊断主要依靠病理组织学及免疫组织化学。     

肾脏上皮样型血管平滑肌脂肪瘤临床病理分析

目的 探讨肾脏上皮样型血管平滑肌脂肪瘤(EAML)的临床病理特点及诊断与鉴别诊断.方法 通过对3例EAML临床病理学观察与免疫组化标记及复习有关文献,讨论其组织学特征及临床生物学行为.结果 3例年龄分别为28、40和32岁,均有腰痛病史,其中1例伴有肉眼血尿,均无结节硬化症.眼观:肿瘤位于肾脏,直径大小分别为6 cm、8 cm和3 cm.3例境界清楚,例2伴囊性变.肿瘤无包膜,2例出血、坏死明显,质脆或韧.镜检:瘤细胞体积大或巨大,呈多边形,胞质丰富,嗜酸性或空泡状,核仁明显,可见核内包涵体,多核巨细胞散在其中.瘤细胞弥漫性或巢团状排列.2例瘤组织出血、坏死明显,呈浸润性边缘.3例未见典型AML图像.免疫组化染色显示瘤细胞HMB-45阳性,SMA和vimentin散在性阳性,CK、EMA和CD10均阴性.结论 EAML是一种单一方向分化的,含有大上皮样细胞的肿瘤,瘤细胞较为特异的表达HMB-45.辅以免疫组化可以与其它肾脏肿瘤相鉴别.     

Clinicopathologic features of renal epithelioid angiomyolipoma: report of one case and review of literatures

Epithelioid angiomyolipoma (EAML) is a rare renal mesenchymal tumor with malignant potential and is frequently associated with tuberous sclerosis complex (TSC). As metastasis of the tumor cells occur early, EAML is considered a potentially malignant tumor type and intrigues further research on it. Under the microscope, we could find the tumor was composed of atypical polygonal cells sheet mixed with classic angiomyolipoma (AML) components such as blood vessels with notable thick vascular walls, smooth muscle-like cells and adipocytes. Immunohistochemical studies showed that epithelioid cells were focally positive for vimentin, melanocytic markers (HMB-45), myoid markers (α-smooth muscle actin), CD34 and CD68; negative for cytokeratin, epithelial membrane antigen, CD10, and S-100. And the Ki67 index showed approximately 3%. Here, we report the morphological and immunohistochemical features of clinically or histologically malignant renal EAML and discuss its diagnosis, differential diagnosis and the prognosis.     

Sclerosing variant of epithelioid angiomyolipoma

Presented herein are two unusual epithelioid angiomyolipomas (AML) displaying prominent stromal sclerosis. Both patients were middle-aged women without a clinical history of tuberous sclerosis. One patient (case 1) had a 2 cm lesion arising in the renal cortex, and another (case 2) had a pararenal retroperitoneal tumor measuring 13 cm. Both tumors were composed of sheets or nests of polygonal epithelioid or short spindle cells having uniform round to oval nuclei and eosinophilic cytoplasm with cords of hyalinized sclerotic stroma between them. The tumor in case 2 had small areas of mature-looking fat cells. Immunohistochemically, epithelioid tumor cells were diffusely positive for actins and desmin in both cases, and melanoma antigen recognized by T cells (MART)-1 was positive in patient 2. Scattered HMB-45-immunoreactive cells were identified in the sclerotic cords of both tumors, but epithelioid tumor cells were essentially negative for HMB-45. The characteristic clinicopathological and immunohistochemical features of the present cases are analogous to a subset of epithelioid AML or sclerosing perivascular epithelioid cell tumors previously reported.     

肝脏血管平滑肌脂肪瘤9例临床病理分析

目的探讨肝脏血管平滑肌脂肪瘤(hepatic angiomyolipoma,HAML)的临床病理特点、诊断及鉴别诊断。方法对9例HAML进行临床病理学观察与免疫组化标记及复习有关文献。结果 9例HAML年龄28～56岁,平均44.2岁,均无结节硬化症。术前影像诊断多数为肝癌。眼观:肿瘤直径1.2～14 cm。肝左叶4例、肝尾状叶3例和肝右叶2例。肿瘤境界清楚,无包膜,质地脆、易碎,可见出血、坏死。镜检:瘤组织由上皮样细胞、平滑肌样细胞、脂肪组织和厚壁血管组成。上皮样细胞体积大或巨大,呈多边形,胞质丰富,嗜酸性或空泡状,核仁明显,可见核内包涵体,偶见多核巨细胞。平滑肌样细胞呈梭形,9例中有4例几乎缺如脂肪组织。瘤细胞弥漫性或巢团状排列。瘤组织出血、坏死明显,呈浸润性边缘。免疫组化标记:瘤细胞HMB-45及Melan-A阳性,SMA和vimentin散在阳性,CK、EMA、AFP均阴性。结论 HAML是一种罕见的间叶组织源性肿瘤,多见于女性,组织学上以单一方向分化的大上皮样细胞为主,瘤细胞较为特异的表达HMB-45及Melan-A。免疫组化标记可与其它肝脏肿瘤鉴别。     

Characteristic cytologic findings of epithelioid hemangioendothelioma: A case report and review of literature

Epithelioid hemangioendothelioma is a rare vascular neoplasm of low-grade malignancy. We observed a case of epithelioid hemangioendothelioma arising in a cervical lymph node. Fine needle aspiration cytology showed several characteristic findings of epithelioid hemangioendothelioma, that is, nuclear inclusion bodies, nuclear grooves, and amorphous material, in addition to physaliform chromatin pattern of a new clue. By reviewing the reports, the prevalence of these is clarified. Complex appearance of these findings is useful in the diagnosis.     

Atypical pleomorphic epithelioid angiomyolipoma localized to the pelvis: a case report and review of the literature

AIMS: Angiomyolipoma is the most common mesenchymal tumour of the kidney. It has been reported in several other sites outside the kidney, mainly in the liver. We report the first case of atypical pleomorphic angiomyolipoma in a man, arising from the pouch of Douglas and extending to the entire abdominal cavity. METHODS: A 17-year-old man underwent a complete resection of a giant abdominopelvic mass. The tissue was formalin fixed and paraffin embedded and 4 micro m thick histological sections were stained with haematoxylin-eosin. Immunohistochemical stains for HMB-45, smooth muscle actin, vimentin, calponin, S100 and desmin were performed. Sections for electron microscopy were also prepared. RESULTS: Microscopic examination revealed a neoplasm composed of pleomorphic epithelioid cells with atypical features, immunoreactive for HMB-45, MART-1, actin, vimentin and calponin, while S100 protein and desmin stains were negative. Ultrastructurally, the tumour cells showed prominent nucleoli, vacuolated cytoplasm, and some premelanosomes. A diagnosis of atypical pleomorphic epithelioid angiomyolipoma was then made. CONCLUSIONS: To date five patients with abdominal epithelioid angiomyolipoma have been described in the literature. All were women. Three of the five patients reported developed metastasis, while our patient is still free of disease at 16 months of follow-up. Clear prognostic pathological features have not been identified.     

伴有TFE3扩增的肾脏上皮样血管平滑肌脂肪瘤

目的探讨伴有TFE3扩增的肾脏上皮样血管平滑肌脂肪瘤(epithelioid angiomyolipoma,EAML)的病理学特征、鉴别诊断及生物学行为。方法对1例伴有TFE3扩增的肾脏EAML进行组织形态学观察、免疫组化染色及荧光原位杂交(fluores-cence in situ hybridization,FISH)检测,追踪随访患者预后,并复习相关文献。结果该例EAML呈片状弥漫分布,瘤细胞呈上皮样改变,细胞形态异型性较大,核分裂象易见。肿瘤侵犯包膜。瘤细胞表达SMA、TFE3、cathepsin K,TFE3基因出现多倍体扩增,未见易位发生。患者第一次手术3个月后肿瘤复发,术后半年腹腔肿瘤广泛侵犯、肺部见转移。结论伴有TFE3扩增的EAML组织学形态及生长方式更具恶性特征,预后更差,与经典型EAML有所不同,需与其他形态学相似的肿瘤相鉴别。     

Renal epithelioid angiomyolipoma: Histopathologic review,immunohistochemical evaluation and prognostic significance

Epithelioid angiomyolipoma (EAML) is considered to be a potentially malignant tumor and requires a differential diagnosis from renal cell carcinoma. In this study, we assessed the clinicopathologic features of renal EAML and evaluated the prognostic significance. Among 78 angiomyolipoma (AML) patients, a total of 5 EAMLs were identified, accounting for 6.4% of the total AML cases. The mean age was 41.4 years, and the average tumor size was 12.7 cm in diameter. Association of tuberous sclerosis complex was identified in two cases. One EAML case showed malignant behavior with local recurrence and distant metastasis. The malignant EAML had a larger tumor size, a higher percentage of epithelioid component and atypical epithelioid cells, ≥2 mitoses per 10 high power fields with atypical mitosis, necrosis, extrarenal extension, and carcinoma‐like growth pattern. Furthermore, the malignant case revealed p53 immunoreactivity and decreased membranous E‐cadherin expression. Pathologic evaluation of adverse prognostic factors will be helpful for risk stratification and prognosis estimation of EAML patients.     

Estrogen receptor is significantly associated with the epithelioid variants of renal angiomyolipoma: a clinicopathological and immunohistochemical study of 67 cases

Perivascular epithelioid cells (PEC) in angiomyolipoma (AML) were recently proposed to be its most common progenitor cells. Histologically, triphasic components were present in various proportions, but were overwhelmingly myogenic in epithelioid variants of AML. Despite histological discrimination, the immunophenotypic profiles between triphasic and epithelioid AML have never been compared. The aim of the present study was to clarify the identity of PEC by using immunoreactivity to estrogen receptor (ER), progesterone receptor (PR), bcl-2 and placenta alkaline phosphatase (PLAP), and to use this information to compare triphasic and epithelioid AML. A total of 33 out of 67 cases of renal angiomyolipoma that underwent surgery were reviewed over the period 1998-2003. Two cases were associated with tuberous sclerosis. Ten patients had other malignant tumors, and three patients had a nodal extension. Immunohistochemistry showed that bcl-2 (59.4%), PLAP (46.9%), HMB-45 (100%) was predominantly localized around vessels. The stem cell markers were absolutely negative in all AML types. The estrogen receptors were positive in 14 cases (42.4%) and the progesterone receptors were positive in five cases. Bcl-2 and both female sex hormone receptors were significantly more frequent in the epithelioid variant of AML than in the triphasic type. Perivascular epithelioid cells express bcl-2, ER, PR and PLAP, and ER could be partly associated with myogenic proliferation.