肝脏血管平滑肌脂肪瘤临床病理分析

目的：探讨肝脏血管平滑肌脂肪瘤（hepatic angiomyolipoma,HAML）的病理形态学、免疫组化特征以及诊断、鉴别诊断要点。方法：对18例HAML病例进行组织学观察,HMB-45、Melan-A、Actin、Desmin、S-100、CK、Hep、AFP等免疫组织化学检测,并结合文献讨论。结果：18例HAML,位于肝左叶6例,右叶9例,双侧3例;镜下肿瘤由畸形厚壁血管、血管周梭形或上皮样细胞和脂肪细胞3种成分不同比例混合存在,可有某种成分的缺如;免疫组织化学检测显示血管周梭形或上皮样细胞HMB-45（＋）,Melan—A（＋）,Actin（＋）,Desmin（＋）,S-100（＋）,CK（-）,Hep（-）,AFP（-）。结论：HAML由多种成分组成,形态变化多样,血管周梭形或上皮样细胞表达HMB45或Melan—A（＋）是诊断HAML的可靠证据。     

慢性肝炎血清Ⅳ型胶原和层粘连蛋白的水平及其临床意义

Serum levels of laminin and type IV collagen of 188 patients with different types of viral hepatitis were determined by RIA. Thirty-five blood donors were served as normal control. The results showed that: (1) higher levels of type IV collagen was found in patients with severe chronic hepatitis, post-hepatitis cirrhosis or subfulminant hepatitis; (2) the level of laminin was obviously increased in post-hepatitis cirrhosis and subfulminant hepatitis; (3) there was a positive correlation between the levels of type IV collagen and laminin as well as gamma globulin, and negatively correlated to serum albumin, no correlation with alanine aminotransferase was observed; (4) detecting rate of hepatic fibrosis was increased with combination of serum laminin and type IV collagen examination; (5) serum type IV collagen was more sensitive than that of serum laminin. The results suggest that determination of serum type IV collagen and laminin are useful in diagnosis of hepatic fibrosis, and combination of the two parameters is recommended.     

肝癌细胞在Ⅳ型胶原裱衬表面的粘附特性

定量描述肝癌细胞和肝细胞在Ⅳ型胶原裱衬表面的粘附特性,探讨细胞粘附力与Ⅳ型胶原裱衬浓度及Ⅳ型胶原与层粘连蛋白复裱衬时ＬＮ复合浓度的相关关系。方法采用分步裱衬的方法制作 有关裱衬表面,即依次在玻璃表面裱衬多聚赖氨酸和Ⅳ型胶原／ＬＮ,最后以牛血清白蛋白作用阻断非特异性粘附。细胞粘附力采用微管吸吮技术进行测定。     

Correlative study on the level of serum type IV collagen, laminin and the diameters of portal veins in patients with viral hepatitis]

The contents of serum Type IV collagen and laminin were detected by radioimmunoassay and the diameters of portal veins were measured by B-mode ultrasound in 102 patients with different type of viral hepatitis. The results show that an increase of levels of Type IV collagen and laminin was found in patients with chronic severe hepatitis and fulminant hepatitis. The levels of Type IV collagen and laminin were obviously increased in the patients who have the diameter of portal vein > or = 12 mm compared with the patients who have the diameter of protal < 12 mm. There was a positive correlation between the levels of Type IV collagen, laminin and the diameters of portal vein.     

4种免疫标记物在恶性黑色素瘤和良性痣中的表达

目的探讨4种免疫标记物在恶性黑色素瘤（malignantmelanoma,MM）及良性痣（benignnevus,BN）中的表达及意义。方法应用免疫组织化学技术检测27例MM、23例BN组织标本中HMB-45、S-100、黑色素A（Melan-A）、B细胞淋巴细胞瘤2（B-celllymphoma-2,Bcb2）的表达情况。结果HM昏45在MM中表达较BN明显升高（P〈0．01）,而S-100、Melan-A、Bcl-2在MM及BN中均呈不同程度阳性表达,无统计学差异。4种标记物在MM中表达无相关性。淋巴结转移性MM中,HMB-45呈阴性表达;在梭形细胞型MM中,Melan-A呈阴性表达。结论HMB-45在良、恶性黑色素肿瘤中有鉴别意义,S-100、Melan-A、Bcl-2可作为黑色素肿瘤的诊断标记物,但需多种标记物联合应用,才能提高黑色素肿瘤诊断的准确率。     

男性盆腔血管周上皮样细胞肿瘤临床病理分析

目的探讨男性盆腔血管周上皮样细胞肿瘤的临床病理特点、诊断要点及治疗。方法应用HE及免疫组化染色对6例男性盆腔血管周上皮样细胞肿瘤进行临床病理分析,并复习相关文献。结果肿瘤主要由上皮样和梭形细胞组成,细胞排列成编织状、巢团状,间质血窦丰富。免疫组化示瘤细胞SMA、Melan—A、HMB-45阳性表达,CD34、CD117、S-100、CgA、Syn均阴性。患者行肿瘤切除术,术后随访至今未见复发和转移。结论男性盆腔血管周上皮样细胞肿瘤少见,与其他部位同类肿瘤相似,手术切除是首选的治疗方法。     

肾上皮样血管平滑肌脂肪瘤3例的临床病理分析

目的探讨肾上皮样血管平滑肌脂肪瘤(EAML)的病理诊断要点、鉴别诊断及预后意义。方法对3例肾上皮样血管平滑肌脂肪瘤(EAML)患者的个案包括临床资料、光镜观察、免疫组化及随访结果进行分析。结果 3例患者其中1例无症状,2例主诉为腰部疼痛;1例术前考虑为错构瘤,2例疑为肾癌,镜下肿瘤主要成分为上皮样细胞,呈巢状分布,局部血管周围见少量平滑肌增生,肿瘤边缘见极少量脂肪组织。免疫组化:上皮样肿瘤细胞黑色素瘤相关抗原HMB45,Melan-A均呈表达阳性,局灶表达SMA,不表达CK、CK7、CK20、RCC、CD10、CD68、S-100、CgA。3例患者随访8～24个月均未见复发。结论肾上皮样血管平滑肌脂肪瘤易与肾细胞癌及其他恶性肾肿瘤相混淆,免疫组化有助于鉴别。     

近端型上皮样肉瘤4例临床病理分析

目的:探讨近端型上皮样肉瘤的临床病理特征、诊断要点和鉴别诊断.方法:分析4例近端型上皮样肉瘤的临床病理资料,观察其组织病理学形态及免疫表型特征,对其进行随访.结果:女3例,男1例.发病年龄33～58 岁.位于会阴部2例,胸壁1例,背部1例.临床均表现为无痛性生长的坚实性孤立性结节状肿块.瘤细胞呈圆形或卵圆形,含有均质的嗜酸性胞质,胞核位于一侧,核仁明显,常见横纹肌样细胞,少见肉芽肿样改变.瘤细胞波形蛋白(Vim)、细胞角蛋白(CK)、CD34、上皮膜抗原(EMA)阳性例数分别为4/4、4/4、2/4、1/4,均不表达MyoD1、S-100蛋白、SMA、HMB-45、desmin.2例死于发病后8个月以内,1例发病4个月后出现肝转移灶,12个月死亡,另1例随访14个月仍生存.结论:近端型上皮样肉瘤的组织病理学特征是:瘤细胞有弥漫性深染嗜酸性癌样胞质,核大,空泡状,核仁明显,常见横纹肌样细胞;瘤细胞共同表达Vim和CK,半数病例瘤细胞表达CD34,部分病例瘤细胞表达EMA;诊断需与一些具有上皮样细胞形态的良恶性肿瘤相鉴别;其生物学行为高度恶性.     

胃血管球瘤临床病理分析并文献复习

目的探讨胃血管球瘤的临床病理特征、诊断和鉴别诊断。方法对4例胃血管球瘤行组织学观察和免疫组织化学分析,并复习相关文献。结果肿物位于胃窦或胃体黏膜下,直径1.5~3 cm。显微镜下肿瘤组织在胃肌壁间呈多结节状分布;结节内肿瘤细胞小-中等大小,圆形、多角形,胞界清楚,胞质丰富红染或空亮,细胞核圆形、卵圆形居中,无明显异型性到轻度异型,核分裂像罕见;瘤组织内富含血管,部分瘤细胞围绕分枝状的血管形成血管外皮瘤样结构;部分瘤细胞呈实性分布。免疫组织化学肿瘤细胞vimentin、平滑肌肌动蛋白（smooth muscle antigen,SMA）、actin及层粘连蛋白（laminin,LN）阳性;CK、CD34、CD117、DOG-1、CgA、Syn、S-100、HMB45、Melan-A等阴性。结论胃血管球瘤非常少见,大多数生物学行为表现为良性。确诊需结合组织病理学形态和免疫表型综合分析,需与胃肠间质瘤及神经内分泌肿瘤等鉴别。     

层粘连蛋白对两株小鼠肝癌克隆细胞系体外附壁及浸润的影响

Ｈｃａ－Ｆ和Ｈｃａ－Ｐ细胞系是从小鼠癌中分离出来的具有不同转移能力的两株克隆。在Ｃ３Ｈ／ｈｅｊ小鼠实验组显示，Ｆ细胞比Ｐ细胞具有较强的侵袭力和淋巴道转移能力。在体外附壁实验中，Ｆ细胞比Ｐ细胞更易贴附于层粘连蛋白上，而且，在后者加上Ⅳ型胶原基质后，Ｆ细胞的贴壁能力更增强；纤维连接蛋白加上Ⅳ型胶原可促进Ｐ细胞贴壁。     

半面痉挛患者面神经损伤的超微结构研究

目的：进一步探讨半面痉挛患者面神经损伤的超微病理特征。方法：随机选取10例半面痉挛患者面神经（男6例，女4例），年龄28-67岁，病程6-30年，透射电镜下观察面神经的超微结构改变。结果：有髓神经髓鞘结构严重变性，板层分离形成透明空泡或网络状，并向轴索内膨出，神经微丝，微管溶解断裂，有大量不规则的溶解灶。髓鞘周的雪旺氏细胞及无髓神经也可见不同程度的溶解，间质中的微血管显著变化。胞饮泡增多，胞质水肿，基底板显著增厚或复层化，严重者可见基底板裸露或内皮细胞变性坏死脱落。结论：有髓神经，无髓神经及微血管的严重损伤，可能是半面痉挛患者面神经的超微结构的特征。     

血清铁和铁蛋白与肝病患者肝纤维化指标的关系

OBJECTIVE: To study the relationship of serum iron and ferritin with the indicators for hepatic fibrosis and hepatic iron overload. METHODS: Liver tissue specimens were obtained from 41 patients with benign (16) or malignant (25) liver diseases by 1 second liver biopsy, and routine microscopic examination was performed after haematoxylin-eosin (HE) and Perl's Prussian staining. Atomic absorption spectrum, radioimmunoassay and enzyme-linked immunosorbent assay were respectively employed to examine the serum levels of iron, ferritin, hyaluronic acid, laminin, human procollagen type , and collagen type . RESULTS: Between patients with benign and malignant liver diseases, significant differences were found in the serum ferritin levels (P < 0.05), but not in serum iron levels (P > 0.05). It was also noted that the levels of the indicators for hepatic fibrosis in patients with benign and early-stage malignant diseases varied significantly from the levels in normal subjects, but these differences were not observed between normal subjects and patients with end-stage hepatic malignancies. Serum iron and ferritin were found to be associated with serum laminin levels (serum iron: r=0.439, P=0.031; serum ferritin: r=0.476, P=0.016), and no iron granules detected in the tissue specimens of hepatocellular carcinoma. CONCLUSIONS: Most of the patients with hepatocellular carcinoma have elevated serum ferritin levels. The serum levels of iron and ferritin are statistically correlated with serum laminin level. Obvious reduction of iron content is typical of hepatic malignant tissues in comparison with the benign tissues, and the reduction in the levels of the indicators for hepatic fibrosis might involve the inhibition of collagen synthesis in the tumor tissues from patients with end-stage hepatocellular carcinoma. Most of the cases of alcoholic fatty liver are complicated by liver iron overload, often marked by serum iron and ferritin levels.     

高糖培养的大鼠肾小球系膜细胞中NO的变化及其对细胞外基质的影响

目的 探讨高糖培养的肾小球系膜细胞中一氧化氮（NO）合成的变化,以及上述变化对细胞外基质的影响.方法 体外培养大鼠肾小球系膜细胞,按干预方法分为正常组（NG组,5.56 mmol·L-1 DMEM）,高糖组（HG组,25 mmol·L-1 DMEM）、一氧化氮供体组（SNP组,5.56 mmol·L-1 DMEM＋100 μmol·L-1 SNP）、NO清除剂组（C-PTIO 组,25 mmol·L-1 DMEM＋200 μmol·L-1 C-PTIO）,以Griess比色法测定培养上清中NO水平;用ELISA法测定培养上清中Ⅳ型胶原、层黏蛋白含量.结果 高糖培养条件下上清中NO增多（P〈0.05）,Ⅳ型胶原、层黏蛋白含量增加（P〈0.01）,应用C-PTIO可显著降低上清中NO的含量,Ⅳ型胶原、层黏蛋白的含量也降低.结论 高糖可诱导大鼠肾小球系膜细胞产生NO,异常增多的NO又可导致系膜细胞中Ⅳ型胶原、层黏蛋白的积聚.     

Pigment epithelium-derived factor as a new diagnostic marker for melanocytic tumors

Pigment epithelium-derived factor (PEDF), a potent antiangiogenetic factor, has been lately known to correlate well with angiogenic and metastatic potentials of tumor cells. We investigated the expression of PEDF protein in various types of human tumor cells by an immunohistochemical technique using a monoclonal antibody. Consequently, we found the significantly frequent and intense expression of PEDF in human melanocytic tumor cells including malignant melanoma as compared to non-melanocytic ones. We evaluated the diagnostic usefulness of anti-PEDF antibody in melanocytic tumors by comparing its immunoreactive sensitivity with that of other conventional melanocytic markers such as S-100 protein, HMB-45 and Melan-A, and found that PEDF possess the equal ability to others on its sensitivity. We finally concluded that PEDF is a useful diagnostic marker for melanocytic tumors, especially malignant melanomas, by its use in combination with other markers.     

Interleukin 1, transforming growth factor beta and messenger ribonucleic acid expression during the course of glomerulonephritis and glomerulosclerosis]

The accumulation of extracellular matrix is a prominent feature of progressive glomerulonephritis and glomerulosclerosis. This study was designed to evaluate the coeffective role of interleukin 1 (IL-1), transforming growth factor beta (TGF beta), and accumulation of collagen IV, fibronectin (FN), laminin (LN) in the progress of glomerulonephritis. Rat mesangial proliferation and sclerosis was induced by injection of anti-thymocyte serum (ATS). Total RNA of glomeruli were extracted from rat kidney at 3, 7, 14, 28, 60 days after ATS administration for northern blots. The results showed that IL-1, TGF beta mRNA expression was 2 to 3 folds higher than in the controls on the 7th to 14th day after injection of ATS. At the same time, there were increased accumulations of collagen IV, FN, and LN. Increased IL-1, TGF beta mRNA expression and accumulation of collagen IV, FN, LN were closely associated with mesangial proliferation, matrix expansion and focal glomerulosclerosis. These results suggest that increased IL-1, TGF beta mRNA expression and accumulation of collagen IV, FN, LN may play an important role in the progression of glomerulonephritis.     

Establishment of an osteoblastic osteosarcoma cell line and effects of cell culture conditions on secretion of matrix metalloproteinases from the cultured osteosarcoma cells

Osteosarcoma cells are useful for investigating bone metabolism as malignant counterpart of osteoblasts. In hematogenous metastases of osteosarcoma cells, the cells need to adjust to various changes in pericellular environment. The changes in pericellular environment may change intracellular environment and consequently the secretion of matrix metalloproteinases (MMPs) which destroy extracellular matrices. In this report, a new cell line, KOS-1, derived from human osteoblastic osteosarcoma was established, and we assumed various culture conditions containing ingredients of the extracellular matrix to make a comparative study on MMPs detected from the culture supernatants. A wide spectrum of MMPs, including MMP-1 and -3 which were increased in the presence of interleukin 1 beta, was detected in this cell line. Production of MMP-1, the enzyme which decomposes types I, II, III and X collagen, by the cells, was increased in the presence of type I collagen. MMP-3 (stromelysin-1) which degrades types III and IV collagen, laminin, fibronectin, proteoglycan, etc. was produced more abundantly in the presence of type IV collagen. MMP-2 (72-kd type IV collagenase/gelatinase A) activity was found to be increased in the presence of gelatin and type IV collagen. The MMPs production in cultured osteosarcoma cells was changed depending on the culture conditions. This indicates that the same osteosarocma cells produce different amounts and kinds of enzymes involved in local infiltration and remote metastases and increase the production of the enzymes most required under a specific environment.     

肾脏上皮样血管平滑肌脂肪瘤的临床特点分析

目的:探讨肾脏上皮样血管平滑肌脂肪瘤（EAML）的临床特点。方法:回顾性分析在2012年6月-2018年11月收治的10例EAML患者的临床资料,对其临床表现、影像特点、病理特征、治疗方法和预后进行分析。结果:10例患者中,女性9例,男性1例,平均年龄36岁,3例在体检时发现,7例有阳性体征。彩超可见囊实性或不均匀实性病灶;CT平扫呈低密度或稍高密度影,可见不同程度的强化;MRI在Ｔ2WI上表现为低信号,呈不均匀强化。8例行肾脏部分切除术,1例行根治性肾脏切除术,1例行肾脏切除术。术后病理8例诊断为EAML;２例病理诊断为肾脏血管平滑肌脂肪瘤（AML）,部分呈EAML。免疫组化染色HMB-45（+）、Melan-A（+）、SMA（+）、CK（-）。术后随访时间7～50个月,平均随访时间28个月,暂未发现复发或远处转移。结论:EAML在临床上较少见,早期症状不明显,确定诊断依靠术后病理HE染色和免疫组化染色。EAML具有恶性潜能,治疗主要以手术切除为主,目前预后尚无统一认识,需要长时间密切随访。     

Alport综合征患者肾小球基底膜超微结构改变与层粘连蛋白的异常分布

目的：观测Alport综合征患者肾小球基底膜(GBM)超微结构改变与GBM上层粘连蛋白α1 (laminin α1)和α5 (laminin α5)分布的关系。方法：1998年1月至2008年7月在北京大学第一医院经肾活检病理确诊为Alport综合征的患者20例,另外选择6例肾肿瘤切除术后患者的正常肾组织作为对照。在透射电镜下测量GBM厚度以及增厚和撕裂的范围。对肾组织石蜡切片进行laminin α1、laminin α5免疫组织化学染色,对其在GBM的分布进行半定量评分：0分为0%,1分为≤25%,2分为25%~50%,3分为50%~75%,4分为≥75%。结果：透射电镜下观察Alport综合征患者的GBM均出现不同程度的增厚和撕裂分层。免疫组织化学显示,正常对照组的laminin α1主要在肾小球系膜区,GBM无显色;laminin α5则沿着GBM均匀显色。Alport患者的laminin α1在肾小球系膜区的分布低于对照组,主要在GBM上显色;laminin α5在GBM上的分布低于对照组;GBM上laminin α1与laminin α5半定量评分呈高度负相关(r=-0.83,P<0.001)。GBM增厚及撕裂范围与laminin α1的半定量评分均呈正相关(分别为r=0.76, P<0.001; r=0.56, P=0.015),与laminin α5的半定量评分均呈负相关(分别为r=-0.59, P=0.010; r=-0.53, P=0.025)。结论：Alport综合征患者GBM中异常分布的laminin α1和laminin α5与GBM增厚和撕裂的超微病理改变有关。     

层粘蛋白和Ⅳ型胶原在乳腺癌的表达

目的：探讨细胞外基质在乳腺癌浸润转移中的作用及意义。方法应用病理形态观察及免疫组织化学技术对50例乳腺癌及23例淋巴结转移灶的层粘蛋白、Ⅳ型胶原蛋白表达进行检测。结果在浸润性乳腺癌中,层粘蛋白和Ⅳ型胶原失去在正常乳腺组织及乳腺良性病变组织中的连续线状表达,表现为间线线状、条索状、碎片状或缺如。层粘蛋白和Ⅳ型胶原的表达与乳腺癌组织学分级、淋巴结转移及病理核分裂间具有显著相关性（P＜0．05）,而与组织学分型、肿瘤大小及瘤栓之间未见相关性。在淋巴结转移灶中,除脉管对Ⅳ型胶原及层粘蛋白呈阳性表达外,也见个别转移癌细胞胞浆对层粘蛋白呈阳性反应,部分转移灶癌细胞间存在着碎片状层粘蛋白表达,癌细胞巢周围没有连续的或间短的线性状表达。结论随着腺癌进展,细胞外基质表达逐渐下调,并与组织学分级、淋巴结转移及病理核分裂密切相关,表明分化越差,病理核分裂像赵多,转移能力越强的肿瘤破坏基底膜的能力越强。癌细胞具有产生基底膜橛物质的能力,可能与肿瘤细胞的浸润活性有关。     

子宫颈血管周上皮样细胞瘤临床病理观察

目的:探讨子宫颈血管周上皮样细胞瘤的临床及病理组织学特征。方法:应用光镜观察及免疫组化染色对1例子宫颈血管周上皮样细胞瘤进行临床病理分析,同时复习相关文献。结果:肿瘤主要由上皮样细胞组成,呈片状排列,细胞间分布较多薄壁小血管,呈网状或裂隙样,免疫组织化学对HMB-45、NSE表达。结论:子宫颈血管周上皮样细胞瘤是一种HMB-45阳性标记的独立真性肿瘤类型,其恶性倾向不明,预后良好。