中枢性原始神经外胚层肿瘤的临床病理分析

目的探讨小脑髓母细胞瘤（ＭＢ）和大脑原发性小细胞肿瘤的组织起源，形态特征及生物学行为的异同。方法采用组织病理学和免疫组织化学方法对２１０例ＭＢ和９例大脑原发性小细胞肿瘤的组织形态特征和标志物表达进行比较研究。结果组织形态学观察显示两者均可向神经元和神经胶质分化。免疫组化标记显示６３２％（６７／１０６例）的ＭＢ和５／８例的大脑原发性小细胞肿瘤可同时表达神经元标志物———突触素（Ｓｙｎ）和神经胶质标志物———胶质纤维酸性蛋白（ＧＦＡＰ）。随访结果显示两者均为高度恶性肿瘤，１年生存率分别为３４６３％和２５６５％，统计分析表明肿瘤有灶状坏死、增殖细胞核抗原标记指数高及术后未进行放疗者预后差（Ｐ＜０００１，Ｐ＜００５，Ｐ＝０．０００１）。结论小脑ＭＢ和大脑原发性小细胞肿瘤的形态特征、标志物表达及生物学行为相同，同意将这类肿瘤归类于原始神经外胚层肿瘤     

胚胎发育不良性神经上皮瘤临床病理观察

目的研究胚胎发育不良性神经上皮瘤（DNT）的临床表现、影像学特点、病理组织学特征及治疗和预后。方法应用光镜、电镜及免疫组织化学染色方法（SP法）对18例DNT进行观察分析,并对其中14例进行了随访。结果患者年龄3～46岁,平均年龄22．8岁,男14例,女4例,主要症状为顽固性癫痫发作,术前癫痫病史最长达17年。MRI检查示病灶T1WI呈低信号,T2WI呈高信号,肿瘤周围无水肿及占位效应。18例肿瘤除2例位于小脑外,其余均位于大脑皮质。本组全切除者10例,大部切除者8例。随访14例,13例生存者中,生存1年4个月-11年,平均已生存5．5年,其中已生存10年以上者2例。术后无1例复发。肿瘤呈结节状分布于大脑或小脑的皮质内,部分累及白质。“特异性胶质神经元成分”为DNT病理形态学特征,是由不同比例的少突胶质细胞样细胞（OLC）、成熟神经元和星形细胞组成,肿瘤有明显的微囊变,单个神经元漂浮在微囊的黏液样基质中,本病多伴有肿瘤周围脑皮质发育不良。免疫组织化学染色示神经元及部分OLC突触素、神经微丝及S-100染色阳性,OLC胶质纤维酸性蛋白染色阴性。电镜示OLC有早期神经元分化、星形细胞分化及少突胶质细胞分化。结论DNT属良性肿瘤（WHOI级）,手术切除即可治愈。应结合临床表现、影像学及病理组织学和免疫组织化学结果确诊DNT。     

小儿室管膜瘤临床病理研究

对２３例小儿室管膜瘤进行了临床病理分析，其中男１７例，女６例，平均年龄８．８岁。２３例中肿瘤位于幕上者７例，幕下者１６例，术前平均病程分别为５．３个月和３个月。依据肿瘤的组织学特点，此瘤可分为乳头型（或粘液乳头型）、上皮型和多细胞型；依据肿瘤分化程度，可分为分化型和间变型室管膜瘤。２３例中有１７例胶质纤维酸性蛋白（ＧＦＡＰ）免疫组化染色呈阳性反应，其余６例为阴性。讨论了小儿室管膜瘤的临床病理特点、影响患者预后的因素及其组织学分类。     

肝母细胞瘤DNA含量,增殖细胞核抗原,P21及p53蛋白的表达

肝母细胞瘤ＤＮＡ含量、增殖细胞核抗原、Ｐ２１及ｐ５３蛋白的表达李代强刘磊一、材料和方法１．临床资料：收集１９８３～１９９３年３５例肝母细胞瘤标本。年龄２８天～７岁，３岁以下占８５．７％。男２１例，女１４例，男女比为１．５∶１。均以肝肿块就诊。血清ＡＦ...     

成人小脑髓母细胞瘤MR成像和NSE表达水平

目的:探讨成人小脑髓母细胞瘤的MRI表现及神经元特异性烯醇化酶(Neuron-Specific Enolase,NSE)特点。方法:对17例经手术病理证实的成人小脑髓母细胞瘤的MR表现进行分析。免疫组化法检测肿瘤组织中NSE表达。ELISA试剂盒检测血清NSE水平。结果 :髓母细胞瘤均位于小脑半球,其中右侧11例,左侧6例,部分累及小脑蚓部,肿瘤形状多不规则,小脑皮髓质同时受累,实质部分多分布性,10例为男性,发病年龄最大者为64岁。肿瘤实性部分与小脑灰质比较,T1WI为略低信号,T2WI为等或稍高信号。髓母NSE表达水平和强度显著高于正常人脑组织细胞瘤(P<0.01),17例髓母细胞瘤中NSE大部分呈强阳性表达。病理学显示光学显微镜下组织是原始的、未分化的肿瘤细胞,核梭形,形成许多菊形团瘤细胞。部分组织体积较小,向髓母细胞分化。局部分化为神经节细胞。结论:成人小脑髓母细胞瘤的MRI表现有一定的特征性,能够与其他小脑肿瘤相鉴别。NSE可作为小脑髓母细胞瘤的神经损伤指标。     

67例颅内星形细胞肿瘤临床病理分析

目的：探讨颅内星形细胞肿瘤的组织类型、分级与复发、预后的关系。方法：对６７例颅内星形细胞肿瘤的临床和病理资源进行统计分析。结果：免疫组织化学标记能帮助鉴定组织类型和颅内星形细胞肿瘤的分化程度。间质性星形细胞瘤和胶质母细胞瘤其术后２年、５年的生存率偏低（Ｐ〈０．００５）。结论：间变性星形细胞瘤和肥胖细胞星形细胞瘤更具恶性,易复发。胶质母细胞瘤在颅内肿瘤中恶性程度最高,预后差。     

肺结核、肺癌患者血清肿瘤坏死因子水平的观察

肺结核、肺癌患者血清肿瘤坏死因子水平的观察叶一秀，王巍，李世平，王安生，李晓明我们于１９９３年１月～１９９４年６月检测了７５例肺结核，２１例肺癌患者的肿瘤坏死因子（ＴＮＦ）水平。对象和方法１．健康组３２名健康体检者，男１８，女１４，平均年龄３４．６岁...     

脑多形性黄色瘤型星形细胞瘤的临床病理观察

目的 探讨多形性黄色瘤型星形细胞瘤（PXA）的临床病理特征、诊断及鉴别诊断、治疗及预后。方法 对南京军区南京总医院1980-2004年间6287例中枢神经系统肿瘤中的15例PXA（0．2％）,以及2例会诊病例,进行临床病理学观察,免疫组织化学SP法检测8种抗体的表达：胶质纤维酸性蛋白、波形蛋白、S-100、上皮细胞膜抗原、突触素、神经微丝、CD68及CD34,获得其中10例的随访资料。结果 患者年龄12～55岁,平均30．8岁,男6例,女11例。主要症状为癫痫发作、头痛、头晕等。肿瘤发生于幕上者16例,占94．1％,其中发生于颞叶者7例,占41．2％。肿瘤大小2—7cm,平均4．3cm,9例有囊性变。除2例会诊病例外,全切除12例,次全切除3例。随访10例,生存8例,生存时间10个月- 13年7个月,平均生存6年,生存10年以上者2例。组织学特征为：单核或多核巨怪瘤细胞、梭形细胞和泡沫样瘤细胞混合而成,肿瘤中有丰富的网状纤维及淋巴细胞浸润,缺乏坏死,核分裂象无或少。胶质纤维酸性蛋白、波形蛋白及S-100蛋白免疫组织化学染色均呈弥散阳性表达,CD34阳性率为77％。1例伴有间变特征的PXA,有较多核分裂象（≥5个／10HPF）。2例有脑实质及血管周围间隙的浸润。1例影像学检查提示肿瘤复发及脑膜播散。结论 PXA属WHOII级肿瘤,肿瘤全切除及组织学为典型性PXA者预后较好,少数PXA可复发及间变。瘤细胞巨大、怪异,容易误诊为WHOⅣ级的巨细胞胶质母细胞瘤,两者的鉴别要点在于PXA部分可见泡沫样瘤细胞,核分裂象无或少,缺乏坏死。瘤细胞CD34的阳性表达有助于PXA的诊断。     

自发性小脑出血14例临床分析

自发性小脑出血１４例临床分析邱争鸣１汪明泉２１临床资料一般资料本文报告１９９３年６月至１９９６年９月１４例小脑出血（其中１０例资料由万县市人民医院提供）病例。１４例中男１１例，女３例，年龄３２～７５岁，其中６０岁以上１１例（７０％），平均年龄６２岁。...     

视网膜母细胞瘤的免疫组化研究

本文旨在研究神经元特异性稀醇化酶(NSE),神经胶质原纤维酸性蛋白(GFAP)和S-100蛋白在视网膜中的分布,视网膜母细胞瘤(Retino-blastoma,简称RB)的组织学起源及与预后的关系。材料取自我院手术切除的RB标本28例,经10%福尔马林固定,石蜡包埋,应用ABC法染色。抗体来自Dako公司。观察结果:GFAP在视网膜中Muller细胞、节细胞胞浆标记阳性,在肿瘤组织中阳性细胞17例分布于     

LIPOSARCOMA

Fifty-two cases of liposarcoma were analyzed clinicopathologically, and were grouped into the following five types: myxoid (28 cases), well-differentiated (14 cases), pleomorphic (4 cases), round-cell (3 cases), and mixed (3 cases). Three of the 28 myxoid tumors showed a pleomorphic pattern resembling that of the myxoid variant of malignant fibrous histiocytoma, and one of the 3 round-cell tumors was considered to be a malignant counterpart of hibernoma. Ages ranged from 22 to 86 years with the average age of 49 years, the average patients with myxoid or round-cell liposarcoma being over 10 years younger than those with well-differentiated or pleomorphic liposarcoma. There was also a variation in the site of predilection of the cases: the great majority of myxoid type tumors occurred in the thigh, popliteal fossa and buttock (23 cases), while the majority of well-differentiated type tumors were seen in the retroperitoneum (10 cases). The rate of local recurrence of the tumor was 46%. Four of the 5 well-differentiated liposarcomas which recurred exhibited dedifferentiated pleomorphic components resembling those in malignant fibrous histiocytoma. The prognosis was more favorable in patients with ordinary myxoid tumors than in those with well-differentiated tumors. The overall relative five-year survival rate was 63%.     

Liposarcoma. A clinicopathologic subtyping of 52 cases

Fifty-two cases of liposarcoma were analyzed clinicopathologically, and were grouped into the following five types: myxoid (28 cases), well-differentiated (14 cases), pleomorphic (4 cases), round-cell (3 cases), and mixed (3 cases). Three of the 28 myxoid tumors showed a pleomorphic pattern resembling that of the myxoid variant of malignant fibrous histiocytoma, and one of the 3 round-cell tumors was considered to be a malignant counterpart of hibernoma. Ages ranged from 22 to 86 years with the average age of 49 years, the average patients with myxoid or round-cell liposarcoma being over 10 years younger than those with well-differentiated or pleomorphic liposarcoma. There was also a variation in the site of predilection of the cases: the great majority of myxoid type tumors occurred in the thigh, popliteal fossa and buttock (23 cases), while the majority of well-differentiated type tumors were seen in the retroperitoneum (10 cases). The rate of local recurrence of the tumor was 46%. Four of the 5 well-differentiated liposarcomas which recurred exhibited dedifferentiated pleomorphic components resembling those in malignant fibrous histiocytoma. The prognosis was more favorable in patients with ordinary myxoid tumors than in those with well-differentiated tumors. The overall relative five-year survival rate was 63%.     

免疫组织化学标记在儿童髓母细胞瘤分子分型中的应用及其临床意义

目的:探讨免疫组织化学标记在儿童髓母细胞瘤分子分型中应用的可行性,分析分子分型相关蛋白的表达与肿瘤临床病理特点及预后的关系.方法:应用组织芯片技术及免疫组织化学Elivision法检测β-catenin,Gli-1,NPR3及KCNA1在40例儿童髓母细胞瘤中的表达,分析根据表达结果行分子分型的可行性及蛋白表达与年龄、性别、肿瘤部位、病理分型及预后的关系.结果:40例肿瘤组织中β-catenin,Gli-1,NPR3及KCNA1的阳性率分别为10％,55％,50％及30％,Gli-1阳性表达与患儿年龄及病理类型相关(P＞0.05).Kaplan-Meier生存分析提示＜3岁年龄组及Gli-1阳性组患儿预后较差(P＜0.05).Cox比例风险回归分析显示患儿年龄、病理分型、Gli-1蛋白及NPR3蛋白表达是儿童髓母细胞瘤的独立预后因子(P＜0.05).22例髓母细胞瘤(medulloblastoma,MB)行分子分型,其中WNT型4例,SHH型10例,3型6例,4型2例,Kaplan-Meier生存分析提示分子分型与预后无关(P＜0.05).结论:p-catenin核阳或核浆阳可作为WNT型髓母细胞瘤的蛋白标志物,Gli-1表达与患儿年龄及病理学类型密切相关,阳性表达提示患儿预后较差,是独立的预后因子之一,但其表达与NPR3及KCNA1的表达有部分重叠,因此尚需结合SHH型、3型及4型髓母细胞瘤的其它分子检测方法进行准确的分子分型.     

Neoplasms of the central nervous system in Norway. III. Epidemiological characteristics of intracranial gliomas according to histology

Data were analysed from 4859 patients with different histological types of intracranial glioma registered by the Norwegian Cancer Registry between 1955 and 1984. Glioblastoma comprised 57.9% of all cases. The second most common primary brain tumour was astrocytoma (19.0%), then mixed glioma (9.2%), oligodendroglioma (7.9%), medulloblastoma (3.1%) and ependymoma (2.9%). A primary brain tumour in a child is approximately twice as likely to be an astrocytoma as a medulloblastoma. The age-specific incidence for glioblastoma increases with age, whereas the incidence of astrocytoma and oligodendroglioma peaks at middle age. Both glioblastoma and astrocytoma showed increased incidence rates over the study period and this was most pronounced in the age-group above 60 years. The prognosis for gliomas varied with age at time of diagnosis, generally being better the younger the patient. For oligodendroglioma patients, survival prospects were independent of age at time of diagnosis. The best prognosis was seen in patients up to 30 years with astrocytoma. Applied in epidemiology, the data indicate that astrocytoma, oligodendroglioma, mixed glioma and ependymoma may be treated as a group which should be separated from both glioblastoma and medulloblastoma.     

Renal tumours in children.

A ten-year series of renal tumours in children up to 14 years of age was collected from the files of the Finnish Cancer Register. After histological evaluation of the material, 74 cases were accepted as primary renal tumours. Fifty-eight of these were typical Wilms' tumours, three rhabdomyosarcomas, six foetal hamartomas and seven unclassified malignant tumours. Wilms' tumours were classified into three types on the basis of the histological differentiation. The total five year survival in the Wilms group was 50 per cent with no difference between patients over and under the age of one year. Of the subtypes of Wilms' tumours the sarcomatous type showed worse prognosis than average; only three out of 16 patients were alive after a five-year follow-up time. None of the patients with foetal hamartoma died of tumour, although two fatalities, connected with the surgery, were recorded. The value of histological classification of Wilms' tumours and the importance of the recognition of the foetal hamartoma is emphasized.     

Does c-erbB-2 expression have a role in medulloblastoma prognosis?

The prognosis of patients with medulloblastoma has remained same for the last two decades. This study evaluated the role of c-erbB-2 expression in medulloblastoma as a prognostic marker. Fifty cases of medulloblastomas were investigated for the expression of c-erbB-2 protein using immunohistochemistry. The expression of c-erbB-2 was correlated with age, histology and disease-free survival (DFS). Thirty-five (70%) tumors were c-erbB-2 positive. Immunoreactivity for c-erbB-2 receptor was observed as mixed cytoplasmic and membrane positivity of tumour cells. The mean DFS in c-erbB-2 positive cases was 19.81 months compared to 48.33 months in c-erbB-2 negative cases. c-erbB-2 positivity was found to be an independent predictor of poor outcome in medulloblastoma (p value < 0.05). No correlation of c-erbB-2 expression was observed with the age of patient and the histological type of tumour. Additionally, c-erbB-4 expression was also evaluated in these tumours. Thirty-three cases showed co-expression of c-erbB-2 and c-erbB-4 proteins. However, c-erbB-4 expression alone was not associated with poor outcome, whereas its co-expression with c-erbB-2 was associated with shorter DFS (p < 0.05).     

中枢神经细胞瘤的临床病理特征和预后

目的：探讨中枢神经细胞瘤（CNC）的临床病理特征及其与预后的关系。方法：应用光镜、电镜检查观察了22例CNC的组织病理、超微结构特点,应用免疫组织化学染色观察了突触素、神经元特异性烯醇化酶（NSE）、Leu-7、胶质纤维酸性蛋白（GFAP）、髓磷脂碱性蛋白（MBP）及增殖细胞核抗原（PCNA)董关CNC的表达情况。结果：本组病例年龄4－14岁,平均27．9岁。肿瘤均发生于脑室。随访18例,14例生存8个月至14年11个月;4例死亡,平均生存时间70．7个月。CNC组织学特点,瘤细胞有明显的核周空晕,呈蜂窝状结构,特征性改变为无核纤维岛。细胞异型、核分裂象及灶性坏死罕见。免疫组织化学染色突触素、NSE、Leu-7阳性表达,GFAP7有MBP阴性反应,PCNA极少数细胞阳性。超微结构特点为圆形瘤细胞可见多量细胞突起,突起内含微管、神经分泌颗粒、透明囊泡及溶酶体样结构。结论：常规光镜检查难以鉴别CNC和少突胶质细胞瘤,免疫组织化学和电镜对确诊CNC很重要。CNC一般预后良好。间变性组织学特点与生物学行为之间的关系仍不清楚。     

Central nervous system tumors among Koreans--a statistical study on 697 cases

A total of 697 cases of intracranial and intraspinal tumors was obtained from the pathology file of Seoul National University Hospital and Children's Hospital during the period of 8 years from 1980 to 1987. These tumors were classified according to WHO classification. This study was performed to understand the recent trend of the relative frequency of the central nervous system tumors among Koreans and to compare it with the previous studies in Korea and other countries. There were 663 intracranial tumors and 34 intraspinal tumors. More common intracranial tumors were pituitary adenoma, meningioma, astrocytoma and medulloblastoma, each representing 23.4%, 20.8%, 11.8%, and 5.6%, respectively. In juvenile age group (under 15 years of age), medulloblastoma, astrocytoma, ependymoma and craniopharyngioma were more commonly encountered to be 25.6%, 21.6%, 13.6% and 12%, respectively. Both sexes were equally affected among adult group, but male preponderance was observed among juvenile group (1.49:1). Nine cases of primitive neuroectodermal tumor, a unique tumor which is not listed in WHO classification, were observed and all of them occurred before the age of 20. There were 27 metastatic tumors. Our previous study encompassing previous 17 years, 1963 to 1979, showed similar overall results except for intraspinal tumors that were more commonly encountered in previous series.     

Paget's osteosarcoma and post-radiation osteosarcoma: secondary osteosarcoma at Middlemore Hospital, New Zealand

Aims: To review the clinical and histological features of our cases of Paget's osteosarcoma and post-radiation osteosarcoma. Methods: A search through the files of the New Zealand Bone and Soft Tissue Tumour Registry was performed, patients were identified and the relevant details were collated. Results: Thirty-one cases of Paget's osteosarcoma and eight cases of post-radiation osteosarcoma were identified. Patients with Paget's osteosarcoma were aged between 48 and 67 years, predominantly female, and axial and appendicular skeleton were equally affected. The outcome was known in 29 cases, with a median survival of 7.25 months and 5 year overall survival of 10%. Patients with post-radiation osteosarcoma were aged between 17 and 68 years, equally male or female, and axial and appendicular skeleton were equally affected. Index lesions included benign or malignant osseous and non-osseous conditions. The average age at diagnosis of the index lesion was 30.1 years and the average latent period was 13.5 years. The outcome was known in all eight cases, with a median survival of 33 months and 5 year overall survival of 38%. Conclusions: Paget's osteosarcoma and post-radiation osteosarcoma are examples of secondary osteosarcoma. The former affects elderly patients, and has a poor prognosis and response to treatment. The later affects a wide age group, and has a prognosis and response to treatment comparable with primary osteosarcoma.