2007年WHO神经系统肿瘤分类（第四版）几个新增肿瘤类型

2007年新修改的WHO关于神经系统肿瘤分类(第4版)共增加了七种新的肿瘤类型,包括血管中心性胶质瘤、毛细胞粘液样星形细胞瘤、垂体细胞瘤、乳头状胶质神经元肿瘤、第四脑室菊形团形成的胶质神经元肿瘤,松果体区乳头状肿瘤和腺垂体梭形细胞嗜酸细胞瘤,学习和掌握这些肿瘤的临床、形态、免疫和遗传学特点,对作好神经系统肿瘤的临床与病理诊断大有裨益。     

16例小脑毛细胞型星形细胞瘤MRI及病理分析

目的 探讨小脑毛细胞型星形细胞瘤的磁共振成像(MRI)影像特点和病理学特征.方法 回顾性分析16例小脑毛细胞型星形细胞瘤的术后病理、术前MRI资料.结果 16例小脑毛细胞型星形细胞瘤中,发生于小脑蚓部11例,小脑半球5例.小脑毛细胞型星形细胞瘤可呈单纯囊肿型、囊肿附壁结节型或瘤囊型.MRI平扫肿瘤境界清楚,无明显瘤周水肿;增强扫描囊壁瘤结节或瘤体部分明显强化,囊壁光滑、强化或不明显强化.显微镜下瘤组织内致密、疏松区双相交替,瘤细胞呈细长梭形,致密区见数量不等Rosenthal纤维,疏松区有微囊样结构、嗜酸性小体形成;肿瘤免疫组化GFAP强阳性.结论 小脑毛细胞型星形细胞瘤MRI影像表现和病理组织学具有特征性,把握其病理特点有助于术前影像诊断.     

乳头状胶质神经元肿瘤临床病理特征

背景与目的:报道1例罕见的乳头状胶质神经元肿瘤(papillary glioneuronal tumor,PGNT),结合文献探讨其临床病理学特征。方法:对1例PGNT的临床表现、影像学特征、病理形态改变和免疫组化标记进行观察分析。结果:患者16岁,临床表现为头痛和偶发癫痫发作。MRI检查见右额顶叶脑实质内有边缘清楚的囊性病灶,囊壁薄,囊腔面见微小突起,囊内充有液体。组织学检查见肿瘤由小圆形细胞组成,类似脑室中央神经瘤瘤细胞,并见星形胶质细胞成分,类似毛细胞性星形细胞瘤样结构。囊腔面瘤细胞排列成特征性的假乳头样结构,中心为血管,表面为单层或多层胶质纤维酸性蛋白(GFAP)阳性的星形细胞。肿瘤与正常脑组织分界清楚,无包膜,靠近正常组织的瘤细胞成片排列,局部有中央神经细胞瘤的无核区结构,瘤细胞对神经元免疫组化Syn、NSE、CgA标记呈阳性反应。肿瘤中见个别中型神经节细胞分化细胞,未见坏死和核分裂相。患者术后追踪8个月,未见肿瘤复发。结论:PGNT是一种新鉴定的神经元-胶质混合性肿瘤实体,属良性脑肿瘤,预后良好。     

低度恶性纤维黏液样肉瘤的临床病理特征

目的:了解低度恶性纤维黏液样肉瘤的临床病理特征.方法:收集2例低度恶性纤维黏液样肉瘤的临床资料,手术切除组织光镜切片观察,另作9项免疫组织化学标记,CKp,EMA,Vimentin,SMA,MSA,CD34,CD99,S-100,CD68.结果:2例男女各1例, 肿瘤由梭形细胞区和星形细胞黏液样区构成,1例有典型的巨形菊形团样结构.肿瘤呈浸润性生长,易复发.免疫组织化学标记瘤组织CKp,EMA,Vimentin,SMA,MSA,CD34,CD99,S-100,CD68阴性,Vimentin阳性.结论:低度恶性纤维黏液样肉瘤的诊断靠病理组织学和免疫组织化学标记,手术切除为主要的治疗方法.     

婴儿促纤维增生型星形细胞瘤1例并文献回顾

目的：探讨婴儿促纤维增生型星形细胞瘤病理形态特征及鉴别要点。方法：报道1例婴儿促纤维增生型星形细胞瘤并结合文献对其组织学和免疫组织化学特点进行研究。结果：本例婴儿促纤维增生型星形细胞瘤头颅CT表现为右侧额骨及顶骨局部骨质缺损，右侧大脑半球多囊性占位性病变。组织学表现为梭形肿瘤细胞，形似纤维细胞或纤维母细胞，排列呈束，编织样。免疫组织化学显示：瘤细胞呈胶质酸性蛋白（GFAP）阳性，波形蛋白（Vimentin）阳性，S-100蛋白阳性。结论：婴儿促纤维增生型星形细胞瘤是一种比较罕见的发生于婴幼儿的脑部肿瘤，根据其临床特点及组织学和免疫组织化学特征可以进行明确诊断。     

低度恶性纤维黏液样肉瘤的临床病理特征

目的：了解低度恶性纤维黏液样肉瘤的临床病理特征。方法：收集2例低度恶性纤维黏液样肉瘤的临床资料,手术切除组织光镜切片观察,另作9项免疫组织化学标记,CKp,EMA,Vimentin,SMA,MSA,CD34,CD99,S-100,CD68。结果：2例男女各1例,肿瘤由梭形细胞区和星形细胞黏液样区构成,1例有典型的巨形菊形团样结构。肿瘤呈浸润性生长,易复发。免疫组织化学标记瘤组织cKp,EMA,Vimentin,SMA,MSA,CD34,CD99,S-100,CD68阴性,Vimentin阳性。结论：低度恶性纤维黏液样肉瘤的诊断靠病理组织学和免疫组织化学标记,手术切除为主要的治疗方法。     

婴幼儿促硬纤维增生性星形细胞瘤临床病理分析

目的探讨DCAI临床病理特点、IHC和鉴别诊断.方法对1例DCAI进行临床病理特征及免疫组化观察.结果患儿女性,17个月,主诉双下肢乏力半个月.颅脑MRI示肿瘤位于后颅窝,边界清,与硬脑膜粘连,囊实性,最大径为5.6cm,压迫同侧小脑半球并长入椎管,囊性与实性区示明显对比增强,组织学上呈双相改变,由肿瘤性星形细胞和促硬纤维增生性间质共同参与,似富于细胞性间叶性肿瘤,需与脑膜瘤鉴别.免疫表型示弥漫性GFAP、S-100蛋白、vimentin强阳性,明确支持瘤细胞为胶质细胞.结论 DCAI是发生于婴幼儿罕见肿瘤,表现为大脑半球巨大性肿块,组织学上瘤细胞呈束状漩涡状排列,形成席纹状结构,象脑膜瘤或纤维组织细胞瘤,需与脑膜瘤、DIG、PXA、胶质纤维瘤鉴别.确诊有赖于独特的临床、影像学、组织学、免疫组织化学及电镜下见到大量广泛一致的基底层物质分布于S-100蛋白(+)GFAP(+)的瘤细胞之间.     

脑星形胶质细胞瘤增殖与分化

 本文应用免疫组化方法在40例呈形胶质细胞瘤中研究PCNA和GFAP两种标记表达，原位观察与比较瘤细胞的增殖与分化。结果表明脑星形胶质细胞瘤中PCNA与GFAP表达率均为100％，PCNA表达强度与肿瘤分级呈正相关（P＜0．05），GFAP表达强度与肿瘤分级呈负相关（P＜0．01）。提示PCNA与GFAP的表达对于判断脑星形胶质细胞瘤的增殖活性，分化程度及判断肿瘤的恶性程度有一定的价值。     

星形细胞肿瘤DEK的表达及其与病理分级的关系

目的:检测星形细胞肿瘤组织中DEK的表达,分析其与病理分级之间的关系。方法:应用免疫组化技术并用图像分析系统检测各级别星形细胞肿瘤组织及脑组织中DEK的表达水平。结果:各组免疫组化染色阳性率分别为:肿瘤组织（86.6%）,其中毛细胞型星形细胞瘤(WHO I级66.7%),弥漫性星形细胞瘤（WHO II级84.2%）,间变性星形细胞瘤（WHO III级93.3%）,胶质母细胞瘤（WHO IV级100.0%）;脑组织（33.3%）。各组免疫组化染色强度分别为:肿瘤组织 23.57±8.12,其中毛细胞型星形细胞瘤14.23±5.31,弥漫性星形细胞瘤19.52±6.68,间变性星形细胞瘤32.12±13.56,胶质母细胞瘤36.92±15.61;脑组织11.61±1.85。DEK在肿瘤组织中的表达显著高于其在脑组织中的表达（P<0.05）。结论:DEK的过表达与星形细胞肿瘤的发生密切相关,并且与肿瘤的病理分级也密切相关;DEK表达改变,为星形细胞肿瘤临床诊断、治疗和预后判断提供了新思路。     

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。     

Coexpression of glial and neuronal markers in the neurocytic rosettes of rosette-forming glioneuronal tumors

Rosette-forming glioneuronal tumor of the fourth ventricle (RGNT) is a new entity in the WHO 2007 Classification of Tumors of the Central Nervous System. RGNT has two components: neurocytic rosettes and low-grade gliomas. Neurocytic rosettes are conventionally described as consisting of uniform neurocytes. However, some studies have reported rosette-forming tumor cells that expressed glial markers such as Olig2. We indicated the expression of glial markers including Olig2, cyclinD1, glial fibrillary acidic protein (GFAP), and platelet-derived growth factor receptor alpha (PDGFRα) in the neurocytic rosettes in our previous study, and we suggested that these tumor cells had a heterogeneous nature. In this study, we used double and triple immunostaining to demonstrate that these tumor cells have both glial and neuronal characteristics. We found that rosette-forming tumor cells coexpressed Olig2/cyclinD1 and synaptophysin. Furthermore, the cores of the rosettes coexpressed GFAP/PDGFRα in the peripheral zone and synaptophysin in the central zone. These findings imply that rosette-forming tumor cells have a similar nature to neuronal-glial progenitor cells, and we believe that the nomination “neurocytic rosette” may be unsuitable given their heterogeneous nature. Our study appears to clarify some of the properties of RGNT tumor cells and may help elucidate the histogenesis of RGNT.     

A reassessment of histologic classification and an immunohistochemical study of 88 retinoblastomas. A special reference to the advent of bipolar-like cells.

BACKGROUND. Despite perpetual efforts of investigators, the histogenesis of retinoblastoma is still in dispute and histologic classification satisfactorily predictive of prognosis does not seem to be in use. METHODS. The authors studied 88 cases of retinoblastoma clinicopathologically and immunohistochemically, paying special attention to the presence of a "bipolar-like cell" element that would be used as one of the criteria for the diagnosis of differentiated retinoblastoma. RESULTS. Twelve cases of retinoblastoma with the bipolar-like cell element in the absence of rosettes and 41 cases of the tumor with rosettes were classified as differentiated retinoblastomas. The other 35 cases without rosettes or bipolar-like cells were classified as undifferentiated tumors. Tumor cells forming rosettes usually had positive results for synaptophysin and neuron-specific enolase (NSE) and negative results for glial fibrillary acidic protein (GFAP) and S-100 protein; however, undifferentiated cells had negative results for these four antibodies. The bipolar-like cells had positive results for synaptophysin and NSE but negative results for GFAP and S-100 protein. Twelve tumors with bipolar-like cells that lacked rosettes showed no optic nerve invasion, and the patients had a significantly better prognosis (100% 5-year survival rate) than 35 patients with undifferentiated tumors (71% 5-year survival rate) (P < 0.01). CONCLUSIONS. The findings support a neuronal origin of the tumor and indicate that, not only the rosettes symbolizing the photoreceptor differentiation, but also other neuronal elements, such as bipolar-like cells, can be used as criteria for histologic classification of retinoblastoma.     

A case of undifferentiated glioma in a 70-year-old woman

The present case involved a 70-year-old woman who was diagnosed with a right cerebral hemorrhage. Excisional surgery of the hematoma was performed. Grossly, a whitish, solid tumor (1×1×0.8cm in size) was recognized in large, polygonal cells and small undifferentiated cells in a jumbled architectural arrangement with a cartilage component. The large, polygonal cell component was conspicuous and somewhat rhabdoid in appearance and appeared to be an astrocytic tumor showing glial differentiation. The small, undifferentiated cell component resembled tumor cells of a primitive neuroectodermal tumor (PNET). Clinical follow-up of the patient for 2 months after the first operation revealed recurrence with rapid growth. A second operation was performed, but the patient died 8 months after the first operation (2 months after the second). Immunohistochemically, the tumor cells suggesting glial differentiation were positive for glial fibrillary acidic protein (GFAP), S-100, neuron-specific enolase (NSE), and vimentin. PNET-like components in the primary tumor were positive for NSE, GFAP, and S-100, and weakly positive for vimentin and synaptophysin. Each tumor cell was negative for epithelial membrane antigen (EMA), keratin, desmin, actin, myoglobin, neurofilament (NF), and MIC2 protein. The recurrent tumor revealed predominantly PNET-like components; however, only a few tumor cells were positive for GFAP. This appearance suggested that this brain tumor might originate from a common multipotential stem cell. Considering its histopathological and immunohistochemical characteristics, the primary tumor was finally regarded as an undifferentiated glioma with dedifferentiation of the glial component in the recurrent tumor.     

An immunocytochemical comparison of glial fibrillary acidic protein,S-100p and vimentin in human glial tumors

Immunostaining patterns of glial fibrillary acidic protein (GFAP), S-100 protein (S-100p) and vimentin were studied using immunohistochemical techniques on 48 paraffin embedded glial tumors. GFAP was positive in all tumor cases except in two oligodendrogliomas. S-100p was found in most astroglial tumors and in half of the oligodendrogliomas. Vimentin was positive in many astrocytomas but in no oligodendrogliomas. Most astroglial tumors showed similar immunoreactivity for GFAP and S-100p. Fibrillary processes, however, showed stronger and more crisp staining with anti-GFAP than with anti-S-100p, whereas cell nuclei were labeled only for S-100p. Vimentin was localised mainly in juxtanuclear positions.In many astrocytomas with different degrees of malignancy co-expression of GFAP, S-100p and vimentin was found. The presence of GFAP and S-100p was not correlated with the degree of differentiation in astrocytomas. Vimentin was more positive in anaplastic astrocytomas but this finding was not statistically significant. It seems that GFAP is a superior marker to S-100p and vimentin in the identification of human gliomas.     

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。     

The prognostic significance of glial fibrillary acidic protein staining in medulloblastoma

Histologic and immunohistochemical properties of 53 medulloblastomas were analyzed with regard to clinical features and survival rate. No correlation was found between survival rate and histologic features of the tumor, such as desmoplastic reaction, number of mitoses, hemorrhages, necrosis, endothelial proliferation, glomerular arrangement, calcifications, rosettes, or oligodendroglial cells. However 82% of the patients with positive glial fibrillary acidic protein (GFAP) staining in numerous tumor cells survived more than 5 years; only 30% survived that long if their tumor cells were GFAP negative (P = 0.0093). This significant difference was not related to the mode of therapeutic protocol used. The authors suggest that GFAP staining may be a useful prognostic tool in medulloblastoma.     

A case of angioglioma composed of astrocytoma with a papillary growth pattern: Immunohistochemical and ultrastructural studies

We report a case of a large cystic astrocytoma associated with arteriovenous malformation in the right cerebral hemisphere of a 16-year-old boy. Neuroimaging showed large abnormal vessels with flow voids and arteriovenous shunt around the cystic lesion. Histologically, the cyst wall was formed by abnormal vasculature and clusters of glial cells forming a papillary growth pattern. The abnormal vasculature consisted of dilated vein-like vessels and medium-sized arteries with incomplete media, and was diagnosed as an arteriovenous malformation. Immunohisto-chemically, glial fibrillary acidic protein (GFAP) decorated both the perikaryon and the processes of the glial tumor cells. They were negative for epithelial membrane antigen (EMA), cytokeratin, and S-100 protein. Ultrastructurally, the tumor cells were rich in intermediate filaments, and neither cilia, microvilli, nor ependymal rosettes were verified. Based on these morphological features and the low MIB-1 labeling index of 0.8%, the glial tumor was diagnosed as astrocytoma, Grade II, according to the World Health Organization (WHO) tumor classification. An association of glioma with various types of vascular anomalies has been designated as angioglioma. A unique feature of the present case, however, is a papillary growth pattern, which is not listed in the current WHO classification of brain tumors. The recognition of the occurrence of such cases would be important in differential diagnosis of papillary ependymoma and choroid plexus papilloma.     

Neuronal and glial proteins in medulloblastomas. I. Immunohistochemical study

Neuronal and glial specific proteins were studied in 35 cases of medulloblastomas, using immunohistochemical methods and antibodies to neurofilament triplet proteins (NF68Kd, NF160Kd, NF210Kd), gamma-enolase, GFAP, S-100 protein, and myelin basic protein. NF160Kd positive cells were observed in 12 of the 35 cases. NF160Kd positive cells were noted in 2, but NF210Kd positive cells were not detected. Gamma-enolase positive cells were observed in 23 cases. GFAP and S-100 protein positive cells were seen in 25 and 28 cases respectively; many of these cells were reactive astrocytes. The myelin basic protein positive cells could not be identified. The expression of neuronal and glial proteins had no relation to the postoperative survival of patients with medulloblastoma, but gamma-enolase may be one factor for evaluating prognosis.     

Pineoblastoma with neuronal differentiation — Immunohistochemical and immunocytochemical studies

Summary Immunohistochemical and immunocytochemical studies were performed on a specimen obtained at operation from a 4 year-old female with intracranially spread pineal tumor in the right temporal lobe. Microscopically, it was a highly cellular tumor, and the cells contained chromatin-rich round or oval nuclei and scanty cytoplasms. There were numerous mitotic figures. Rossetes were not detected. Both immunohistochemical staining of paraffin-embedded sections and immunocytochemical staining of short-term cultured cells disclosed a subgroups of cell positive for NSE and 68K-NF (neurofilament). No cells were positive for GFAP, S-100 protein or HCG. From these findings, the tumor was thought to be pineoblastoma, which is an undifferentiated tumor that rarely shows divergent differentiating potential. This is the first report of pineoblastoma with the differentiation only toward the neuronal line confirmed by the analysis of immunohistochemistry and immunocytochemistry.     

Solitary intracranial plasmacytomas

We present two cases of an unusal localised extramedullary plasmacytoma with a long survival period: a 42-year-old woman with left temporal and a 25-year-old woman with left parietal intracranial plasmacytoma. The tumor masses were totally removed in both patients and their histological, histochemical (PAS and methyl green pyronin positive granules) and immunohistochemical (positive light chains mostly lambda, and negative stains for GFAP, NSE and S-100 protein) properties showed that the tumor tissues consisted of monoclonal population of plasma cells. Our cases were diagnosed as solitary cerebral plasmacytomas since the presence-of underlying multiple myeloma has been ruled out by the clinical, laboratory, radiographic and immunological investigations. Postoperatively the patients were given 40 Gy to the whole cranium and additional 20 Gy focused on the tumor site. Complete remissions were achived 7.5 and 5.5 years, respectively.