脑节细胞胶质瘤8例临床病理学分析

摘 要：［目的］ 探讨节细胞胶质瘤的临床病理学特点。［方法］ 应用组织病理学及免疫组织化学观察8例脑节细胞胶质瘤的组织学特点,并结合文献探讨其病理形态及诊断、鉴别诊断。［结果］5例伴头痛,3例伴慢性癫痫发作;6例呈实性,2例呈囊实性,1例伴钙化。6例完全切除,2例未完全切除;8例组织学上均表现为肿瘤性胶质细胞和发育不良的节细胞;7例为WHO I级,1例为WHO Ⅲ级。免疫组化胶质成分表达GFAP、S-100,而节细胞表达Syn、NSE和CgA。Ki-67增殖指数较低,5例CD34阳性,3例局灶表达CD34。［结论］ 节细胞胶质瘤为生长缓慢的良性肿瘤,大部分属于WHO I级,常见部位为颞叶。主要根据组织学和免疫组织化学进行诊断,完整切除后可治愈。     

间变性节细胞胶质瘤复发为幕上原始神经外胚层肿瘤一例报告并文献复习

背景与目的：间变型节细胞胶质瘤非常少见,恶变总是发生在胶质成分。目前已有少量病例显示神经元成分的恶性转化。本文报道一例原发瘤为间变性节细胞胶质瘤,术后8个月复发为幕上原始神经外胚层肿瘤的病例。方法：观察并分析原发瘤和复发瘤的病理形态特征和免疫组化标记。结合文献讨论间变性节细胞胶质瘤转变为幕上原始神经外胚层肿瘤的可能机制。结果：患儿8岁。镜下见第一次切除的左颞叶肿瘤：部分区域肿瘤细胞密集分布。细胞较小,核染色较深,部分瘤细胞呈小片状,细胞稍大,核圆形或多角形,染色质淡。肿瘤组织中另可见散在或聚集向神经元分化的不同阶段的肿瘤细胞。细胞较大,有明显淡红染的胞浆,胞核空泡状,有核仁。有的似分化较成熟的节细胞。网状染色见瘤组织中纤维组织明显增生。免疫组化结果显示：GFAP灶性（＋）、NSE（＋）、S100（＋）、Nestin（＋）、VIM（＋）、Des（＋）。似神经元分化的大细胞则有NSE和S-100的阳性表达。病理诊断：伴有纤维增生的间变性节细胞胶质瘤。术后8个月左颞部复发肿瘤中除了仍见明显的纤维组织增生外,另见小或中等大小的肿瘤细胞密集排列,瘤细胞更异型,核分裂多见。未见较成熟分化的细胞。免疫组化显示：GFAP灶性（＋）、S100（＋）、NFP（＋）、Neuronal class III beta-tubulin（＋）。提示肿瘤细胞向神经元和胶质成分双向分化。病理诊断幕上原始神经外胚层肿瘤。结论：节细胞胶质瘤可以出现胶质和神经成分的恶性转化。     

形成菊形团的胶质神经元肿瘤临床病理观察

目的:探讨形成菊形团的胶质神经元肿瘤(RGNT)的临床病理学特征.方法:对1例发生于第四脑室形成菊形团的胶质神经元肿瘤的临床表现、影像学、组织形态学、免疫组化以及分子病理学等结果进行分析,并结合文献探讨其诊断与鉴别诊断要点.结果:组织学上表现为神经细胞和毛细胞样星形细胞并存,神经细胞较小、一致,围绕神经毡结构形成菊形团样结构或围绕血管形成假菊形团样结构.胶质成分形态学符合毛细胞星形细胞瘤.免疫组化显示:神经毡样基质呈Syn、NSE阳性,GFAP、CgA阴性.神经细胞核S-100、Neu-N阳性.胶质成分呈GFAP、S-100阳性,Ki-67<2%.结论:形成菊形团的胶质神经元肿瘤是混合性胶质神经元肿瘤中的少见类型,2007年列入第四版中枢神经系统WHO,是WHO I级的低级别胶质瘤.组织学上以胶质成分及围绕神经毡呈菊形团排列的神经细胞成分为主,免疫组化有鉴别诊断意义.     

MDM2和p53在反应性及肿瘤性星形胶质细胞中的表达

目的：检测MDM2和p53蛋白在星形胶质细胞反应性增生与星形胶质细胞瘤中的表达，探讨二者在胶质瘤形成和发展中的作用及其相关性。方法：应用组织芯片和免疫组化染色技术检测正常脑组织、星形胶质细胞反应性增生、低级别（Ⅰ-Ⅱ级）和高级别（Ⅲ-Ⅳ级）星形胶质细胞瘤中MDM2和p53蛋白的表达情况。结果：正常脑组织中MDM2和p53蛋白均呈阴性表达；反应性增生组、低级别肿瘤组及高级别肿瘤组中MDM2蛋白的阳性率分别为32.7％（16／49）、59．2％（29／49）、80．0％（40／50）；p53蛋白的阳性率分别为27．3％（12／49）、57．1％（28／49）、82．0％（41／50）。二者阳性表达率均随着病变恶性程度的增加而升高，MDM2和p53在各实验组间的比较差异均有统计学意义（P〈0．05）；且MDM2和p53表达密切相关（P〈0．05）。结论：MDM2和p53在星形胶质细胞反应性增生及星形胶质细胞瘤中均呈不同程度的过度表达，且随着病变恶性程度的增加表达水平增高，MDM2扩增和p53突变是胶质瘤发生的早期事件，二者的联合检测可能会对星形胶质细胞反应性增生与低级别胶质细胞瘤的鉴别诊断以及星形胶质细胞瘤的早期诊断提供一定的依据。     

乳头状胶质神经元肿瘤报告一例及文献复习

背景与目的：自中央性神经细胞瘤和胚胎发育不良性神经上皮瘤(dysembryoplastic neuroepithelial tmors，DNT)被确认以来，脑室外小圆细胞组成的肿瘤受到重视，又报道了乳头状胶质神经元肿瘤(papillary glioneuronal tumor，PGNT)和节细胞神经细胞瘤等新肿瘤。本文报道一例PGNT，复习其临床病理特征和文献，对此类肿瘤的特征进行探讨。方法：观察一例新病例的临床和病理表现，包括HE和免疫细胞化学染色并复习文献进行讨论。结果：本例(男性，32岁)肿瘤位于大脑顶叶，瘤组织由乳头状结构及少突胶质样细胞和上皮样细胞区交织构成，其中有小圆神经细胞、中等大的神经细胞和大的双核节细胞。免疫细胞化学染色中显示大多数瘤细胞和神经毯呈现S-100和Syn阳性反应，有些瘤细胞呈现GFAP阳性反应，本例病理诊断为PGNT。对肿瘤的临床和病理特征作了讨论，认为PGNT为较良性的肿瘤。结论：PGNT为一种低级别的胶质神经元肿瘤，以特异性乳头状胶质血管结构为特征。免疫细胞化学染色做NSE，Syn和GFAP等有助与中央性神经细胞瘤、DNT及其它胶质瘤相鉴别。PGNT这一新的肿瘤实体有可能成为神经元和混合性神经元胶质肿瘤中的一员。     

306例颅内肿瘤的临床病理分析

目的：探讨颅内肿瘤组织类型与临床表现，预后之间的关系，为颅内肿瘤的防治提供一定的依据。方法：回顾分析306例颅内肿瘤的临床资料。结果：颅内肿瘤男女发病率相近。常见类型为胶质瘤和脑膜瘤。临床表现较复杂，术前诊断主要依靠CT、MRI等。免疫组化标记有助于病理诊断。结论：胶质瘤和脑膜瘤术后有复发倾向，复发次数越多，预后越差。免疫组化标记能更加明确肿瘤的组织类型、分化程度及预后。     

囊性肾透明细胞癌1例及文献复习

目的：探讨囊性肾透明细胞癌的临床病理特征、诊断及鉴别诊断。方法：对1例囊性肾透明细胞癌进行光镜观察,行组织化学及免疫组化染色,并复习文献。结果：肿瘤细胞主要由透明细胞组成。癌细胞免疫表型Ckpan、EMA、CEA呈阳性表达,CD68阴性。结论：囊性肾透明细胞癌是一种罕见的肾癌病理类型。其诊断依赖组织病理学和免疫组化标记。     

胶质瘤p^27Kipl、bcl-2和PCNA蛋白的表达

目的：探讨胶质瘤p^27Kipl，bcl-2和PCNA蛋白表达与肿瘤恶性程度、细胞增殖活性、凋亡程度的关系。声去采用免疫组化染色S-P法检测66例不同级别的胶质瘤p^27Kipl，bcl-2和PCNA蛋白的表达。结果：在66例胶质瘤甲，p^27Kipl表达18例(27％)，bcl-2表达20例(30％)，PCNA表达51例(77％)。p^27Kipl蛋白表达率随着胶质瘤级别升高而减少．bcl-2蛋白表达率随肿瘤级别升高而相应增加；PCNA表达随胶质瘤级别升高阳性反应强度增加；但Ⅰ、Ⅱ与Ⅲ级和Ⅳ级组间无显著性差异。结论：p^27Kipl蛋白表达的缺失可能与胶质瘤的发生有关；bcl-2基因可能间接抑制细胞凋亡而与胶质瘤的分型、细胞的增殖活性以及潜在的临床行为无直接关系；PCNA的表达与星形胶质细胞瘤的恶性行为有关。     

高分级胶质瘤的放化综合治疗

胶质瘤是起源于神经胶质细胞的肿瘤，主要包括星形细胞肿瘤、少突胶质细胞肿瘤和混合性胶质细胞肿瘤。星形细胞瘤是胶质瘤中最常见的类型，其中的间变性星形细胞瘤又称恶性星形细胞瘤，属WHOⅢ级。临床常将恶性星形细胞瘤和胶质母细胞瘤统称为恶性胶质瘤，而将Ⅲ级胶质瘤（如间变性星形细胞瘤，间变性少突胶质细胞瘤）、胶质母细胞瘤、胶质肉瘤等统称为高分级胶质瘤。     

Pleomorphic xanthoastrocytoma as a component of a temporal lobe cystic ganglioglioma: a case report

We report a case of pleomorphic xanthoastrocytoma (PXA) as a component of a ganglioglioma in a 13-year-old Japanese boy. Magnetic resonance imaging showed a large cystic lesion with an enhanced mural nodule of the left temporal lobe. Microscopic examination of the tumor showed that it was composed of two distinct neoplastic components: dysplastic ganglion cells and a PXA. There were gradual transitions between the two neoplastic components, and the PXA constituted the gliomatous component of the ganglioglioma. The PXA component showed spindle-shaped and pleomorphic large cells with lipidized cytoplasm. The tumor cells were surrounded by numerous reticulin fibers. Immunohistochemically, the ganglion cells were negative for glial fibrillary acidic protein (GFAP), but showed positive staining for a 70-kDa neurofilament protein, synaptophysin, and NeuN. In contrast, PXA cells were positive for GFAP but negative for neuronal markers. Our case is therefore histologically classified as ganglioglioma with PXA as the glial component. These results suggested that PXA and ganglioglioma share a common origin and that the combination of PXA-ganglioglioma would be positioned along the spectrum between PXA and ganglioglioma. Alternatively, these results may support the hypothesis that PXA originates from glioneuronal progenitor cells capable of generating astrocytic and neuronal cell types.     

Anaplastic oligodendroglioma with ganglioglioma-like maturation

Neuronal differentiation of oligodendroglioma has been demonstrated by immunohistochemical and ultrastructural examinations in recent studies. However, oligodendrogliomas displaying a complete neurocytic morphology or even gangliocytic differentiation are rare. We describe a case of anaplastic oligodendroglioma that was characterized by the presence of ganglion cells in a 40-year-old-male. Histologically, the tumor was mainly composed of classical oligodendroglioma cells. The most exceptional finding of this tumor was the presence of ganglion cells and intermediate-sized ganglioid cells. Immunohistochemical analysis revealed that these cells were positive for Olig2 and negative for glial fibrillary acid protein (GFAP). Synaptophysin and microtubule-associated protein 2 (MAP2) were mainly detected in the ganglion cells. Fluorescence in situ hybridization analysis (FISH) revealed the deletion of the 1p and 19q chromosome arms in both the oligodendroglioma cells and ganglion cells. The R132H mutated isocitrate dehydrogenase 1 (IDH1) protein was detected by immunohistochemistry and direct DNA sequencing. The morphological, immunohistochemical, and genetic features of the tumor suggested a diagnosis of anaplastic oligodendroglioma, and this tumor was considered to be a rare form of oligodendroglioma displaying ganglioglioma-like maturation. FISH and mutant IDH1 examinations are useful diagnostic tools for the differential diagnosis of this tumor, i.e., ganglioglioma with anaplastic oligodendroglial features.     

Intramedullary gangliogliomas: clinical features, surgical outcomes, and neuropathic scoliosis

Intramedullary spinal cord gangliogliomas are rare tumors composed of glial components and ganglion cells. These gangliogliomas are generally considered as slow-growing tumors, corresponding histologically to WHO grade I or II. There are few reports of large case series of intramedullary spinal cord gangliogliomas from a single center. We retrospectively reviewed a consecutive series of 18 patients with pathologically diagnosed ganglioglioma. Clinical manifestations, radiological features, treatment and follow-up data, and concomitant scoliosis were investigated. The mean age at diagnosis was 27.5 years, with a slight female predominance. The primary clinical symptoms were sensorimotor deficits. Magnetic resonance (MR) imaging manifestations varied considerably. Some associated, but not necessary, features were found, such as young age at onset, large tumor dimension, and bony changes. Scoliosis was observed in seven patients. Remnant tumor progression was observed in five patients during the follow-up period, and no deaths occurred. The last neurological evaluation showed functional improvement from preoperative status in five patients. Differential diagnosis of ganglioglioma based on MR images alone is challenging, but the combination of some characteristic features can be helpful. An accurate diagnosis of ganglioglioma depends on pathological criteria. Despite the benign course of ganglioglioma, considerable growth may affect its resectability and prognosis. The extent of resection should be meticulously planned, and the potential risk of recurrence and neurological deterioration should be evaluated. The concomitant scoliosis is noteworthy.     

24 例软组织透明细胞肉瘤的病理组织学及免疫组化观察

 目的 探讨透明细胞肉瘤的临床病理特点及诊断与鉴别诊断要点。方法 观察24例透明细胞肉瘤的病理形态及免疫组织化学特征。结果 显微镜下肿瘤主要由胞质丰富而透明的多角形或梭形细胞组成，其被纤维组织分隔呈大小不等的巢状或柬状。部分病例中可出现多核巨细胞、细胞外黏液样物质、裂隙、细胞内黑色素颗粒、出血坏死及囊性变。免疫组织化学方面：肿瘤中Vimentin、S-100、HMB45、CK的阳性表达率分别为100％（24／24）、91．66％（22／24），70．82％（17／24）、16．66％（4／24），而EMA均为阴性表达。结论 透明细胞肉瘤预后不良，属于中度恶性肿瘤。     

后肾腺瘤临床病理特征及文献复习（附12例）

目的:探讨后肾腺瘤（metanephric adenoma,MA）的临床病理特征、诊治、预后及其鉴别诊断。方法:回顾性分析12例MA的临床病理特征、免疫组化结果及治疗预后。结果:12例后肾腺瘤均为境界清楚的肿块;肿瘤细胞形态温和,小而密集,排列成腺泡状、乳头状、分枝小管状,间质不明显;细胞丰富,核圆形、卵圆形,无核仁,染色质细腻,胞浆稀少,淡染,少见核分裂象;部分可见砂粒体样钙化。免疫组织化学染色 WT-1、CD57、PAX8、Vimentin、PAX2阳性,CK7、CD10、CD117阴性。结论:后肾腺瘤为一种罕见的肾脏上皮源性良性肿瘤,临床特征不明显,具有独特的组织病理学特征,免疫组化染色对其诊断具有重大意义,治疗以手术为主,完整切除预后良好。     

Anaplastic (malignant) ganglioglioma arising from heterotopic grey matter nodules

Summary Anaplastic ganglioglioma is a rare malignant neoplasm. The short clinical course and the anaplastic histological features are characteristics of malignant ganglioglioma. Immunohistochemistry is necessary for the final diagnosis. The MRI appearances are described here for the first time in literature. Nodular grey matter heterotopias had been demonstrated and the patient developed an anaplastic ganglioglioma two years later, on the same place where the grey matter heteropias had been seen. This finding supports the hypothesis that these tumours may arise from grey matter nodules.     

Rap2b基因真核表达载体构建及其对NIH3T3细胞P38通路的影响

目的构建pcDNA3.1-Rap2b真核表达载体,以外源基因Rap2b转染NIH3T3细胞,以了解该基因对NIH3T3细胞AKT、ERK、JNK和P38信号转导通路的影响,为探讨该基因在人肺癌发生中的作用提供实验依据。方法构建人Rap2b真核表达载体pcDNA3.1-Rap2b并稳定转染NIH3T3细胞,利用Western blot方法对转染的细胞进行AKT、ERK、JNK和P38磷酸化蛋白及总蛋白表达水平检测。结果转染pcDNA3.1-Rap2b质粒的细胞与转染空质粒pcDNA3.1的细胞相比,其AKT、ERK、JNK磷酸化蛋白表达水平差异无统计学意义（P＞0.05）,而P38磷酸化蛋白表达水平明显上调（P＜0.05 ）。结论Rap2b基因外源性高表达可能对P38通路有活化作用,对JNK、ERK、AKT通路无活化作用。     

Outcome and prognostic features in anaplastic ganglioglioma: analysis of cases from the SEER database

Anaplastic ganglioglioma (AGG) are rare central nervous system tumours. Patient and treatment factors associated with outcome are poorly defined and limited to small retrospective case series and single case reports. Using the Surveillance, Epidemiology, and End Results (SEER) cancer registry, we investigated potential clinicopathological factors that can affect outcome in patients with anaplastic ganglioglioma. Patients with anaplastic ganglioglioma diagnosed between 1973 and 2007 were identified from the SEER database. Kaplan-Meier survival analysis and Cox models were used to examine the effect of variables on overall survival. The variables analysed included patient age at diagnosis, gender, race, tumour location, uni-focal or multi-focal tumour, surgical resection and the use of adjuvant radiotherapy. Fifty-eight patients were identified, with a median age at diagnosis of 25.5 years. Ninety-three percent of patients underwent surgery and 36% received adjuvant radiotherapy. The median overall survival was 28.5 months. The most common tumour site was the temporal lobe (27%). Univariate and multivariate analysis identified surgery and uni-focal disease as important predictors of overall survival. Adjuvant radiotherapy did not influence overall survival. This study represents the largest analysis of anaplastic ganglioglioma to date. Furthermore it also emphasises the role of national tumour databases for furthering our understanding of rare brain tumours and determining management options.     

钠氢交换蛋白抑制剂Cariporide对MCF-7/ADR细胞化疗敏感性影响机制研究

目的 肿瘤细胞膜内外pH值的改变对于肿瘤的侵袭、转移密切相关,通过细胞内外的H+/Na+交换维持细胞内环境的稳态,细胞的凋亡水平与化疗药物耐药的发生密切相关.本研究观察钠氢交换蛋白1(sodium hydrogen exchange 1,NHE-1)抑制剂 Cariporide(CP)对人乳腺癌细胞MCF-7/ADR增殖、凋亡和对多柔比星(adriamycin,ADR)敏感性影响,并探讨其相关作用机制.方法 以终浓度分别为3、6、9、12、60和100 μg/mL的CP处理MCF-7和MCF-7/ADR细胞48 h,以终浓度分别为1、5、50和100 μg/mL的ADR以及2种药物联用分别处理MCF-7/ADR细胞24、48 h,CCK8法检测其对细胞的增殖影响,Graphpad prism 5分别计算单用及药物联用时的IC50,CompuSyn软件计算2种药物的联用指数(CI);采用流式细胞术检测药物单用及联用后对细胞凋亡率的影响;RT-PCR法检测MDR-1、NF-κB等相关核转录因子的表达;蛋白质印迹法检测CD44、MDR-1、NF-κB p65、Caspase-3/9和Bcl-2的表达.结果 CP对乳腺癌细胞有增殖抑制作用,呈剂量及时间依赖性,CP与ADR联用可以明显降低ADR对MCF-7/ADR细胞的IC50值,其值由(82.45±5.86) μg/mL下降至(42.2±2.03) μg/mL,t=7.57,P<0.05.流式细胞术检测结果显示,对照组、单用ADR组、单用CP组、药物联用组细胞的凋亡率分别为(4.02±0.19)%、(6.34±0.39)%、(9.55±0.47)%和(15.12±0.65)%,F=666.2,P<0.001.荧光定量PCR结果表明,单用CP及CP联用ADR组MDR-1、NF-κB mRNA 表达均下降.蛋白质印迹法结果显示,单用CP及CP联用ADR耐药相关蛋白CD44、MDR-1表达下调,NF-κB p65表达下调,抑凋亡蛋白Bcl-2表达下降,凋亡相关蛋白cleaved Caspase-3和cleaved Caspase-9表达上调.结论 CP可以增加MCF-7/ADR细胞对ADR的敏感性,可能是通过下调耐药相关蛋白的表达,活化凋亡相关蛋白,诱导细胞凋亡,从而有效逆转肿瘤细胞的耐药.