外阴血管肌纤维母细胞瘤1例并文献复习

目的:分析外阴血管肌纤维母细胞瘤(Angiomyofibroblastoma,AM阳)的临床病理特征.方法:结合文献复习,对1例罕见的AFMB进行临床特点、病理形态及免疫组化研究,并探讨其鉴别诊断.结果:AMFB在肉眼上表现为境界清楚的结节;显微镜下,该瘤呈现为在富于水肿和富于薄壁血管的背景中见细胞密集区和细胞稀疏区交替存在,瘤细胞梭形、胖梭形或上皮样,围绕血管排列,间质中见散在肥大细胞和淋巴细胞浸润.免疫组化瘤细胞表达Vimentin、CD34、CD99、bcl-2和PR,不表达Desmin、S-100、ER、EMA、AE1/AE3以及SMA.结论:AMFB是一种罕见的良性肿瘤,手术切除后未见局部复发,罕见恶变,可经外科完全切除而治愈.主要应与外阴侵袭性血管粘液瘤鉴别.     

肾黏液小管状和梭形细胞癌1例报告并文献复习

目的：探讨肾黏液小管状和梭形细胞癌的临床病理特点及诊断、鉴别诊断。方法：对1例肾黏液小管状和梭形细胞癌进行临床病理学及免疫组织化学分析。结果：肾黏液小管状和梭形细胞癌患者临床无特殊表现,肿块与周围肾组织分界清楚。镜下观察：肿瘤呈不规则管状结构、间质呈黏液状,瘤细胞呈立方形或梭形、胞浆嗜酸,核形规则,核分裂少见。免疫组化染色AE1／AE3、EMA、Vimentin阳性表达,S-100、Desmin、SMA、CD34、HMB45、CD10、CD15、CgA、Syn、NSE、CD99阴性表达,Ki-67（3％）。结论：肾黏液小管状和梭形细胞癌为少见肿瘤,明确该肿瘤的组织起源及病理特征,对于病理与临床诊断和鉴别诊断有重要意义。     

卵巢子宫内膜样间质肉瘤临床病理观察

目的探讨卵巢子宫内膜样间质肉瘤的发生机制、临床病理特征、诊断与鉴别诊断及预后。方法 对1例卵巢子宫内膜样间质肉瘤进行光镜、免疫组化检测, 并复习有关文献。结果 肿瘤组织由形似增生期子宫内膜间质细胞的小细胞组成, 瘤细胞呈卵圆形或短梭形, 胞浆稀少, 肿瘤细胞呈漩涡状围绕似子宫内膜螺旋小动脉的厚壁小血管。免疫组化示瘤细胞表达Vimentin和CD10, 不表达Calretinin, α-inhibin, H-caldesmon, CD99, CD117, CD34, desmin, SMA and CK(AE1/AE3)。结论 卵巢子宫内膜样间质肉瘤在临床为一惰性生长的恶性肿瘤, 非常罕见。其鉴别诊断主要包括子宫内膜间质肉瘤累及卵巢(直接侵犯或转移)和卵泡膜细胞-纤维瘤。     

肾脏黏液性管状和梭形细胞癌的临床病理学探讨

目的探讨肾脏黏液性管状和梭形细胞癌的临床病理学特征及诊断、鉴别诊断要点。方法对2例肾脏黏液性管状和梭形细胞癌进行临床病理学及免疫组织化学分析。结果2例肾脏黏液性管状和梭形细胞癌患者，临床症状无特异性。肿瘤与周嗣组织分界清楚。镜下肿瘤呈不规则管状结构伴黏液样间质，瘤细胞呈立方形或梭形，胞浆嗜酸，核呈低级别。无核分裂或少见，无病理性核分裂。免疫组化染色EMA、AE1／AE3、Vim均阳性表达，3413E12弱或灶区阳性，S-100、SMA、HMB45、CD15、CgA及SYN均阴性，NSE在1例中有弱的表达。结论肾脏黏液性管状和梭形细胞癌是极为罕见的，明确该肿瘤的组织起源及病理特征，对于诊断及鉴别诊断有重要意义。     

肾黏液小管状和梭形细胞癌1例报告并文献复习

目的:探讨肾黏液小管状和梭形细胞癌的临床病理特点及诊断、鉴别诊断.方法:对1例肾黏液小管状和梭形细胞癌进行临床病理学及免疫组织化学分析.结果:肾黏液小管状和梭形细胞癌患者临床无特殊表现,肿块与周围肾组织分界清楚.镜下观察:肿瘤呈不规则管状结构、间质呈黏液状,瘤细胞呈立方形或梭形、胞浆嗜酸,核形规则,核分裂少见.免疫组化染色AE1/AE3、EMA、Vimentin阳性表达,S-100、Desmin、SMA、CD34、HMB45、CD10、CD15、CgA、Syn、NSE、CD99阴性表达,Ki-67(3%).结论:肾黏液小管状和梭形细胞癌为少见肿瘤,明确该肿瘤的组织起源及病理特征,对于病理与临床诊断和鉴别诊断有重要意义.     

梭形细胞变异型弥漫性大B细胞淋巴瘤1例报道及文献复习

目的：探讨梭形细胞变异型弥漫性大B细胞淋巴瘤的临床病理特征、病变性质及鉴别诊断要点。方法：对1例梭形细胞变异型弥漫性大B细胞淋巴瘤的组织形态特征、免疫组化表型，结合临床预后进行分析。结果：1例23岁女性患者在半年内先后发生结肠肿瘤和乳腺肿瘤。HE切片显示肿瘤细胞弥漫性浸润肠壁和乳腺组织，瘤细胞有异型性和核分裂，并伴有显著的梭形细胞特征。免疫表型显示瘤细胞CD45 ，CD20 ，CD45RO-，CD30-，Vim ，Des-，SMA-，S-100-，CK-，EMA-。患者1年内死于肿瘤。结论：本例梭形细胞变异型大B细胞淋巴瘤是一种罕见的高度恶性肿瘤，在缺乏免疫组化标记的情况下易误诊为肉瘤或低分化癌。     

肠系膜淋巴结滤泡树突细胞肉瘤1例报告并文献复习

目的探讨滤泡树突细胞肉瘤的临床病理特征、诊断和鉴别诊断。方法对1例肠系膜淋巴结滤泡树突细胞肉瘤进行光镜和免疫组化观察,结合文献进行讨论。结果镜下肿瘤由旋涡状、束状排列的胖梭形、卵圆形或多边形瘤细胞和大量混杂的淋巴细胞组成。免疫表型瘤细胞表达CD35和CD23,弱阳性表达CD68、S-100和EMA;淋巴细胞表达CD45RO和CD3。结论淋巴结滤泡树突状细胞肉瘤是一种罕见的免疫辅助细胞中度恶性肿瘤,其诊断依靠组织病理学和免疫组化,治疗以手术切除为主,必要时辅以化疗和(或)放疗。     

混合性血管内皮瘤1 例报告及文献复习

 目的 探讨混合性血管内皮瘤临床病理特点及免疫组化表型。方法 对1例混合性血管内皮瘤病理组织学及免疫组化表型中的CD31、CD34、VⅢ因子、VWF、PCK、Vimentin、EMA进行观察并复习相关文献。结果 巨检：送检骨骼肌带筋膜，肌间见31个灰白色结节，直径0．6～2．5cm，切面灰白色鱼肉状。镜检：瘤组织成分复杂，有网状型血管内皮瘤、上皮性血管内皮瘤及梭形细胞血管内皮瘤成分。免疫组化表型为血管来源肿瘤。结论 混合性血管内皮瘤属极其罕见低度恶性肿瘤，成分多样。免疫组化表型有助于其诊断分析。     

肺指突性树突状细胞肉瘤伴颈部淋巴结转移1例及文献回顾

目的：探讨指突性树突细胞肉瘤的临床病理特征及诊断、鉴别诊断。方法：对1例原发于肺部的指突性树突细胞肉瘤伴颈部淋巴结转移病例进行组织学和免疫组化研究。结果：肺部穿刺标本中，瘤细胞呈圆形、卵圆形及不规则的梭形等，弥漫杂乱排列，细胞核深染伴明显异型，可见核分裂像及少量瘤巨细胞核。颈部淋巴结中，瘤细胞呈圆形、卵圆形、梭形及不规则形。瘤细胞胞质较少。并见瘤巨细胞核；部分瘤细胞核淡染，核呈泡沫样，并见核仁。瘤细胞间散在小淋巴细胞、浆细胞、组织细胞并伴有嗜酸性粒细胞浸润。免疫组化结果：瘤细胞呈AI—K、CIM5、CIM5RO、CD68、CD163、HLA—Dr、Lysozyme、Fascin、S-100、Vimentin阳性；CD15、CD30局灶阳性；α—SMA、Actin、CNA4．2、N—Cadherin、CD1a、CD11c、CD20、Keratin、MelanA等均阴性。结论：指突性树突细胞肉瘤较为罕见，诊断必须依赖免疫组化检测。应与组织细胞肉瘤、滤泡性树突细胞肉瘤、朗格汉斯细胞肉瘤、恶性纤维组织细胞瘤、转移性恶性黑色素瘤等其他软组织肉瘤鉴别。     

结外指突状树突细胞肉瘤1例临床病理观察并文献复习

目的：探讨脾脏指突状树突细胞肉瘤的诊断要点及其鉴别诊断。方法：对1例脾脏指突状树突细胞肉瘤进行组织病理学、免疫组化染色,并随访。结果：肿瘤原发于脾脏并累及肝脏,于确诊4月后死亡。镜检：肿瘤组织呈席纹状、旋涡状或杂乱排列,瘤细胞卵圆形、梭形,胞质少,核卵圆形或短梭形,染色质少,部分有核仁,分裂象多见。瘤细胞S-100、CD68散在阳性、Vimentin和LCA阳性;CD21、CD35、CD1a等均阴性。结论：指突状树突细胞肉瘤极为罕见,容易漏诊和误诊,熟悉其临床病理特点,依靠免疫组化和电镜检查可确诊。     

Clinic Pathological Features of Angiomyofibroblastoma in Vulva

Objective： To analyze the clinicopathological features and differential diagnosis of angiomyofibroblastoma （AMFB） of the vulva. Methods： Two cases of AMFB were examined by light microscopy and immunohistochemical study and to discuss the clinicopathological features and differential diagnosis of AMFB with the reference to the literature. Results： Tumors were all circumscribed, and 〈5 cm in diameter. Microscopically, the tumors were composed of spindle or polygonal cells that were cellularly or hypocellularly arranged with perivascular accentuation in a mucoid or fibrocollagenous background. The tumors contained numerous small- to medium-sized blood vessels, which were characteristically thin walled. Immunohistochemically, two cases of tumor cells were positive for vimentin, SMA, CD34（＋） and FⅧ（＋）. Desmin and MSA were positive in one case; Cytokeratin, S-100, CD31 were negative in both. Conclusion： AMFB is a rare, benign soft tissue tumor that occurs in the genital tract of adult women. The origin remains unclear, but it is suggested that an origin from a perivascular pluripotent stem cell that is capable of myofibroblastoma differentiation. Angiomyofibroblastoma should be differentiated from other neoplasms of the vulva such as aggressive angiomyxoma, superficial angiomyxoma and cellular angiofibroma.     

Aquaporin-4 expression in primary human central nervous system lymphomas correlates with tumour cell proliferation and phenotypic heterogeneity of the vessel wall

No literature data are available concerning the expression of aquaporin-4 in primary central nervous system lymphomas and the relationship between aquaporin-4 expression and the morphological characteristics of blood vessels. Here, we have investigated this relationship in 24 human diffuse large B-cell primary central nervous system lymphomas by means of immunocytochemistry and confocal laser microscopy. Results have shown that: (i) a high aquaporin-4 expression correlated with a high Ki-67 index and aquaporin-4 marked tumour and endothelial cells in cytoplasm and plasma membranes, while aquaporin-4 expression was low in tumour areas with a low Ki-67 index where few tumour cells were positive to aquaporin-4, and endothelial cells showed aquaporin-4 expression on their abluminal side. (ii) Different type of cells participated in vessels formation: CD20(+) tumour cells and factor VIII(+) endothelial cells; aquaporin-4(+) tumour cells and CD31(+) endothelial cells; CD20(+) and aquaporin-4(+) tumour cells; glial fibrillary acidid protein(+) endothelial cells surrounded by glial fibrillary acidic protein(+) tumour cells. Overall, these data suggest the importance of aquaporin-4 in primary central nervous system lymphomas due to its involvement in cerebral oedema formation and resolution and tumour cell migratory activity, and have documented that tumour microvasculature in lymphomas is extremely heterogeneous, confirming the importance of neoangiogenesis in the pathogenesis of lymphomas.     

Circulating endothelial cells and angiogenic serum factors during neoadjuvant chemotherapy of primary breast cancer

Circulating endothelial cells (CECs) as well as bone-marrow-derived endothelial precursor cells (EPC) play an important role in neovascularisation and tumour growth. To study the impact of neoadjuvant chemotherapy on the amounts of CEC and their precursor cells, mature CEC and their progenitors were quantified by flow cytometry in peripheral blood of breast cancer patients during anthracycline and/or taxane based neoadjuvant chemotherapy and subsequent surgery in comparison to age-matched healthy controls. Cell numbers were tested for correlation with serum levels of angiopoietin-2, erythropoietin, endostatin, endoglin, VEGF and sVCAM-1 as well as clinical and pathological features of breast cancer disease. Circulating endothelial cells were significantly elevated in breast cancer patients and decreased during chemotherapy, whereas EPC (CD34+/VEGFR-2+) as well as their progenitor cell population CD133+/CD34+ and the population of CD34+ stem cells increased. Concomitantly with the increase of progenitor cells an increase of VEGF, erythropoietin and angiopoietin-2 was observed. These data suggest that chemotherapy can only reduce the amounts of mature CEC, probably reflecting detached cells from tumour vessels, whereas the EPC and their progenitors are mobilised by chemotherapy. Since this mobilisation of EPC may contribute to tumour neovascularisation an early antiangiogenic therapy in combination with chemotherapy could be beneficial for the success of cancer therapy.     

子宫上皮样平滑肌瘤临床病理分析

目的 探讨子宫上皮样平滑肌瘤临床病理特征和生物学特性.方法 观察2例子宫上皮样平滑肌瘤的临床、病理组织学及免疫组织化学特点,KRAS基因突变检测分析并复习文献.结果 2例均由胞浆丰富嗜酸的上皮样肿瘤细胞组成.1例体积巨大,大小为20 cm×17 cm×8 cm,镜下未见坏死及核分裂相;1例属多发性子宫平滑肌瘤,其中仅有...     

软组织血管纤维瘤的临床病理学特征分析

目的 软组织血管纤维瘤(soft tissue angiofibroma,STAF)为新近报道的软组织肿瘤,对其临床及组织学形态尚未完全明了.为此,有必要收集更多的病例,以深入探讨其临床病理学特征、鉴别诊断及生物学行为.方法 收集2008-01-05-2016-07-14湖南省人民医院(2例)、佛山中医院(3例)、深圳市龙华新区中心医院(1例)和抚顺市新抚区中医院(1例)共7例STAF,对其临床特征、病理形态和免疫学表型进行分析.结果 7例病例中男3例,女4例,年龄20～61岁,中位年龄为43岁.临床多表现为无痛性肿块,分别位于后颈部头皮下、左膝关节、右外踝、右肘部、前额皮下、左足背及右大腿.肿块部分与周围组织及关节囊有粘连.肿块均完整切除,术后随访4个月至8年,无1例复发.肿瘤直径2～12 cm,境界清楚,切面灰白、灰黄,质地韧或者硬,部分切面有光泽带黏液感.瘤组织主要由大量血管及短梭至卵圆形核的梭形细胞组成,分布于比例不等的黏液样或者胶原化的基质中,梭形细胞形态温和、大小相对一致,局部可见核的不典型性和核的退变,核分裂＜1/10 HPF.瘤内血管主要由大量薄壁分支状血管构成,也可见较大的厚壁血管及呈鹿角样形态的血管外皮瘤样结构,伴有肥大细胞及慢性炎症细胞浸润.组织学上需要与腱鞘纤维瘤、黏液性纤维肉瘤和低度恶性纤维黏液样肉瘤等鉴别.免疫组化检测结果显示,7例患者肿瘤均弥漫表达Vimentin,血管内皮细胞表达CD31、CD34及FLI 1,显示肿瘤中血管的结构和分布特征,SMA在3例患者中局限性表达,DES在4例患者中局限性表达,Ki-67＜1％,AE1/AE3、MSA、Bcl-2、3catenin、Calponin、CD99、Myogenin和MyoD1均为阴性,Ki-67阳性指数为＜1％.结论 STAF是一种好发于肢端的无痛性缓慢增长的具有独特形态学特征的良性软组织肿瘤,主要由程度不等的梭型纤维母细胞及显著分支状血管构成,形态学土需与富含梭型纤维母细胞及血管的多种病变如腱鞘纤维瘤、黏液性纤维肉瘤、低度恶性纤维黏液样肉瘤等相鉴别.临床上手术完整切除可治愈.     

CD133 identifies perivascular niches in grade II–IV astrocytomas

The aim of the present study was to investigate the localization and distribution of the putative brain tumour stem cell marker CD133 in formalin fixed paraffin embedded astrocytomas. A retrospective analysis of 114 grade II, III and IV astrocytomas was undertaken. The immunohistochemical expression of CD133 in paraffin sections was analysed using morphometry. In all grades, CD133 was expressed on tumour and endothelial cells. Tumour cells were found in perivascular niches, as dispersed single cells and in pseudopalisade formations around necrosis. There was no correlation between the mean volume fraction of CD133⁺ niches and all CD133⁺ tumour cells and tumour grade. However, the volume fraction of CD133⁺ blood vessels increased significantly from 0.4% in diffuse astrocytomas to 2.2% in glioblastomas. Neither of them was related to patient survival. Double immunofluorescence stainings showed that the CD133⁺ niches both contained CD133⁺ cells with and without co-expression of the intermediate filament protein marker nestin, and only few CD133⁺/MIB-1⁺ proliferating cells were found. In conclusion, a CD133⁺ perivascular stem cell-like entity exists in astrocytomas. CD133⁺ tumour vessels may play an important role in a brain tumour stem cell context, while CD133 alone does not appear to be a specific tumour stem cell marker related to patient survival.     

New sarcomatoid cancer cell line SAR-HCV established from a hepatitis C virus-related liver tumour lesion

A sarcomatoid carcinoma cell line (SAR-HCV) was established from a malignant liver lesion of a patient infected with hepatitis C virus. SAR-HCV cells were successfully xenografted in SCID mice. Vimentin was strongly positive in cultured SAR-HCV cells, the primary tumour lesion and the xenografts. Hepatocyte paraffin 1 protein and certain cytokeratin markers, CK8, CK18 and AE1/AE3 were not detected in cultured cells, but were focally positive in the tumour lesion and xenografts, suggesting that this cancer cell line preserves some features of hepatocyte differentiation when grown in vivo. HLA class I, N-cadherin, vascular endothelial growth factor, CD44, and heat-shock protein 70 were moderately expressed in this cell line. Spectral karyotyping analysis revealed a nearly triploid karyotype, 34-63<3n>, XXY[12] with complicated genetic abnormalities of chromosomal structure in all metaphases examined. This cell line will be useful in further studying hepato-sarcomatoid carcinoma cells and in understanding carcinogenesis and epithelial-mesenchymal transition in hepatitis C virus-related liver tumour.     

小腿软组织促纤维组织增生性小圆细胞肿瘤临床病理观察(附1例)

目的:探讨促结缔组织增生性小圆细胞肿瘤(DSRCT)的临床病理特点及免疫组化表型及鉴别诊断。方法:对1例DSRCT患者的临床特征、组织学形态及免疫组化表型进行研究,并复习有关文献。结果:DSRCT由成巢的小圆细胞组成。核深染、浆少。纤维组织增生性间质围绕肿瘤细胞巢,可伴透明变性或黏液样变。间质内可有明显血管:毛细血管丛,较大厚壁血管。提示是对肿瘤的增生性反应。核分裂像多。免疫组化多种表型,可表达上皮性、肌性和神经性分化CKAE1/AE3(+)、Vim(+)、Des(+)、NSE(+)、CD99(+)、EMA灶(+)、Myoglobin(+)等。结论:DSRCT为罕见的、侵袭力强的高度恶性肿瘤,瘤细胞具有神经、间叶和上皮标记复合表达。主要累及儿童和年轻男性,一般表现为广泛的腹腔浆膜受累,发病年龄20-30岁。95%发生在腹腔内,常位于后腹膜、盆腔、网膜和肠系膜。临床上位于腹腔外的病例非常罕见,主要在胸腔和睾丸旁区域,个别病例发生在肢体,头颈部和大脑。侵袭性高,预后差,70%因广泛转移而亡。     

Scanning electron microscopy study of the blood supply of human colorectal liver metastases

AIMS: The failure of hepatic artery directed treatment of colorectal liver metastases may reflect a major portal venous contribution to tumour blood supply. This study provides ultrastructural details of the blood supply of colorectal liver metastases and their association with the portal vein and hepatic artery. METHODS: Resected liver specimens from six patients with colorectal liver metastases were examined by histology and scanning electron microscopy (SEM), following vascular resin casting. RESULTS: Nine metastatic colorectal adenocarcinomas were identified. The main feature of all tumours on SEM was direct communication between hepatic sinusoids and tumour vessels. A direct portal venous connection with tumour vessels was observed in a single specimen, whilst a direct arteriole connection was not identified. CONCLUSIONS: It appears that both the hepatic artery and portal vein contribute to the blood supply of colorectal liver metastases through sinusoidal connections with tumour specific blood vessels. SEM provides useful additional information on the morphological features of tumour vasculature.     

VEGF expression by mesenchymal stem cells contributes to angiogenesis in pancreatic carcinoma

Little is known about the factors that enable the mobilisation of human mesenchymal stem cells (MSC) from the bone marrow into the blood stream and their recruitment to and retention in the tumour. We found specific migration of MSC towards growth factors present in pancreatic tumours, such as PDGF, EGF, VEGF and specific inhibitors Glivec, Erbitux and Avastin interfered with migration. Within a few hours, MSC migrated into spheroids consisting of pancreatic cancer cells, fibroblasts and endothelial cells as measured by time-lapse microscopy. Supernatant from subconfluent MSC increased sprouting of HUVEC due to VEGF production by MSC itself as demonstrated by RT-PCR and ELISA. Only few MSCs were differentiated into endothelial cells in vitro, whereas in vivo differentiation was not observed. Lentiviral GFP-marked MSCs, injected in nude mice xenografted with orthotopic pancreatic tumours, preferentially migrated into the tumours as observed by FACS analysis of green fluorescent cells. By immunofluorescence and intravital microscopic studies, we found the interaction of MSC with the endothelium of blood vessels. Mesenchymal stem cells supported tumour angiogenesis in vivo, that is CD31(+) vessel density was increased after the transfer of MSC compared with siVEGF-MSC. Our data demonstrate the migration of MSC toward tumour vessels and suggest a supportive role in angiogenesis.