成人幕上原始神经外胚叶肿瘤

目的:分析总结成人幕上原始神经外胚叶肿瘤(SPNETs)的临床、病理及影像学表现,降低误诊率,提高诊治率。方法:对我院神经外科收治的9例经病理学证实的成年幕上原始神经外胚叶肿瘤患者的临床特点、诊治经过、影像学及病理特点进行回顾性分析。结果:肿瘤位于额叶外、枕叶外生长各2例,大脑镰旁1例,侧脑室旁2例,枕叶、额叶皮质下生长各1例。在影像学、肿瘤生物学和病理学上有一定的共同特征。所有病例均接受手术治疗,8例镜下全切,术后均行全脑、全脊髓X线放疗36Gy(23.4￣39.6Gy),其中2例术后存活超过2年;次全切1例术后2个月肿瘤增大。结论:成人SPNET具有一定的影像学、肿瘤生物学和组织病理学共性,综合分析临床和影像学特征有助于和其他颅内肿瘤性疾病鉴别,提高诊治率。手术结合术后放疗可以延长患者生存周期。     

幕上原始神经外胚瘤五例

目的探讨幕上原始神经外胚瘤(supratentorial primitive neuroectodermal tumor,SPNET)的临床表现、影像和病理特征。方法对5例经病理证实的SPNET的资料,分析其临床表现、影像学组织病理学特征、治疗经过及预后。结果 5例中3例为儿童,2例为成人,主要临床表现为颅内高压症状。CT和MRI检查示肿瘤为幕上肿块,呈类圆形或分叶状,边界清楚,2例有钙化灶,3例无瘤周水肿。病理结果提示肿瘤组织主要由小圆形细胞组成,部分为梭形细胞,细胞密集成巢,由纤维结缔组织分隔成小叶状,核染色质颗粒状,核仁不明显,多数可见核分裂象,形成Homer Wright菊形团。结论 SPNET的影像学和组织病理学表现均有一定特征,影像学及临床表现相结合有助于术前与其他肿瘤相鉴别,提高诊断准确性。     

幕上原始神经外胚叶肿瘤临床病理学初探

目的 探讨原发于颅内幕上的原始神经外胚叶肿瘤（PNETs）的临床病理特征、组织学发生及生物学行为。方法 采用组织病理学、免疫组化标记对9例原发于幕上的PNETs进行研究。结果 肿瘤组织主要由小圆形细胞组成．部分为梭形细胞．细胞密集成巢，由纤维结缔组织分隔成小叶状，核染色质颗粒状，核仁不明显，多数可见核分裂象，形成Homer Wright菊形团。免疫组化标记显示神经特异性烯醇化酶阳性9例，突触素阳性5例，部分瘤组织中波形蛋白、胶质纤维酸性蛋白散在阳性；神经微丝蛋白、白细胞共同抗原、细胞角蛋白呈阴性。结论PNETs属胚胎源性神经上皮肿瘤，具高度恶性，其诊断除了病理形态学观察外，免疫组化检查必不可少。     

幕上原始神经外胚叶肿瘤的临床病理学

原始神经外胚叶肿瘤（primitive neuroeclodermal tumor,PNET）是起源于神经外皮组织的恶性肿瘤，由Hart和Earle于1973年首先描述。由于该肿瘤分化程度差，多呈原始未分化状态，在常规切片诊断上有一定困难，常需与其它小细胞恶性肿瘤鉴别，我们对9便幕上PVET的临床特点，光镜下和免疫组织化学的特征进行了分析总结。     

幕上神经外胚叶肿瘤临床分析(附4例临床报告及文献复习)

目的 探讨幕上神经外胚叶肿瘤的l临床特点及治疗方法.方法 对我院2005年1月～2007年1月收治的4例幕上神经外胚叶肿瘤的临床资料进行回顾性分析.结果 4例患者1例位于顶叶,1例位于丘脑,1例位于额叶,1例位于双额叶及胼胝体.CT显示为高或等密度影,增强后可强化.MRI为长T1长T2信号,或混杂信号,增强后可强化.2例在显微镜下全切,1例次全切,1例部分切除.病理诊断为原始神经外胚叶肿瘤.术后行放化疗,术后3个月～10个月复发.结论 幕上原始神经外胚叶肿瘤为高度恶性肿瘤,愈后差,手术后应行放化疗.     

儿童幕上原始神经外胚层肿瘤

目的 探讨儿童幕上原始神经外胚层肿瘤(ST-PNET)的临床特征、治疗策略和预后.方法 回顾性分析2001年6月至2006年12月问12例经组织病理学证实的儿童ST-PNET的临床资料,并进行随访.结果 男性6例,女性6例,平均年龄6.5岁.8例为脑内肿瘤,4例为脑外肿瘤.11例患者获得随访,随访时间11个月至5年,死亡10例的术后平均生存时间:未放化疗(3例)、单独放疗(3例)、放疗或加化疗(7例)分别为5.0,21.7和23.3个月,后者的3年生存率为14.3%.结论 儿童ST-PNET少见,影像学和免疫组织化学分析有助于诊断.治疗应以手术切除加放疗为主,辅助化疗,但预后极差.     

外周原始神经外胚叶瘤误诊分析

目的 探讨外周原始神经外胚叶瘤(pPNET)的病理诊断要点及误诊原因.方法 收集11例外周原始神经外胚叶瘤,观察其组织学特点,并进行免疫组织化学分析.结果 所有病例均未能明确诊断(省会诊中心会诊后确诊),组织学检查该肿瘤具有一定的形态学特点如大小一致的小圆形细胞,核圆,肿瘤细胞均表达CD99,并不同程度表达NSE、SYN、cgA.结论 外周原始神经外胚叶瘤是一种很少见的肿瘤,病理特点缺乏特异性,仅靠组织形态很难明确诊断,结合免疫组织化学染色结果,可与其他肿瘤区别.     

椎管内、颅内多发原始神经外胚叶肿瘤1例报告

男．46岁．因“胸背部疼痛八年．双下肢麻木两月余”于2002年7月22日入院。查体：左T10、右L2平面以下感觉过敏。MRI示T3～T8椎管内多发异常信号。诊断首先考虑转移瘤，但不排除神经纤维瘤及神经来源之恶性肿瘤的可能。2002年7月29日手术，见T1—Ty水平髓外硬脊膜下散在8个大小不一的肿瘤，主要位于脊髓背侧，鱼肉样，质软，血供丰富，边界清晰．无明显包膜。     

成人幕上原始神经外胚层肿瘤3例报道及文献复习

目的 总结成人幕上原始神经外胚层肿瘤（PNET）的诊治经验。方法 回顾性分析3例经病理证实的成人幕上PNET患者临床资料,并结合相关文献进行分析。结果 本组3例患者中,1例发病1月后死亡,1例确诊后适形调强放疗2年后死亡,1例手术切除。结论 对于进展快、病灶最长径在6 cm,且肿瘤边界清晰、周边水肿不明显的患者,应考虑为PNET可能;PNET预后差;对放疗敏感;必要时做全脑、全脊髓放疗。     

幕上原始神经外胚层肿瘤3例报告

幕上原始神经外胚层肿瘤(supratentorial primitiveneuroectodermal tumor,SPNET)是神经系统胚胎性肿瘤中的一种类型,为罕见的颅内恶性肿瘤。我科近期收治了3例SPNET患者,现报告如下。病例1,男性,66岁,因头痛1年、右眼球疼痛2w入院。查体:右侧眼球略突出,触痛明显,余无明显阳性     

椎管内原始神经外胚层肿瘤

目的探讨原发于椎管内的原始神经外胚层肿瘤（PNET）的诊断与治疗。方法回顾6例椎管内PNET的临床资料，分析其影像学、病理学特点及治疗方法。结果6例椎管内肿瘤有3例化于硬脊膜下，1例局限于硬膜外，1例自椎间孔由硬膜外生长至椎管外，1例累及髓内、椎体、椎旁软组织。全部病例行手术治疗，全切除2例，近全切除2例，活检2例。术后均行HE染色及免疫组化病理检查。术后随访的5例患者中单纯放疗2例，单纯化疗1例，放化疗均进行共3例，均存活。结论椎管内PNET影像学表现多样，诊断困难。病理学检查是诊断的依据。手术切除范围可根据肿瘤生长特点灵活掌握。放化疗对肿瘤有效。但尚无统一的治疗方法。     

Supratentorial primitive neuroectodermal tumor in children: clinical features, treatment outcome and prognostic factors

To investigate clinical features, treatment outcome and prognostic factors of pediatric supratentorial primitive neuroectodermal tumors(ST-PNETs), 28 ST-PNET cases were retrospectively analyzed. The prognostic importance of age, sex, size of tumor, M stage, extent of surgical resection, histological features, immunohistochemical labelling indices (Ki-67, p53), and apoptotic index were assessed. The mean age at diagnosis was 6.8 years, and the male-to-female ratio was 18:10. The presenting symptoms in 22 cases were increased intracranial pressure and focal neurological deficits. Gross total resection was achieved in 17 cases, near-total (>90%) resection in 3, and subtotal in 7; biopsy was performed in 1 case. The mean duration of follow-up was 37 months. For 25 patients who completed planned adjuvant therapy, the 3-year survival rate was 73%. Univariate analysis showed that the presence of tumor necrosis (P=0.002) and extent of resection (P=0.04) correlated with survival. Patients with a high Ki-67 labelling index (>10%) tended to have shorter survival (P=0.095). In multivariate analysis, tumor necrosis showed statistical significance(P=0.03). Received: 20 September 1998 Revised: 16 November 1998     

三例貌似脑炎的幕上原始神经外胚层肿瘤的临床病理分析

目的分析幕上原始神经外胚层肿瘤(s-PNET)的临床、影像学和病理特点,以期早期诊断,早期治疗,从而改善预后。方法回顾分析3例临床上貌似脑炎的s-PNET的临床资料、脑脊液细胞学、头颅核磁共振和脑活检组织病理学特点。结果本文3例患者起病相对较急,主要表现颅高压的症状和局灶神经系统症状,如单肢或偏侧肢体无力,或癫痫发作等。例1脑脊液细胞学可见异形细胞,符合肿瘤细胞。3例患者影像学上病灶边界不清晰,无明显强化。随病情发展,病灶局部扩大或者蔓延至对侧,甚至出现脑脊液播散,并且局部囊性变。3例肿瘤组织绝大部分均由原始幼稚的小细胞构成,弥漫浸润性生长,可见Homer Wright菊形团形成。3例肿瘤细胞一致性表达CD99、Syn、NSE、Nestin和S-100,散在表达CgA、GFAP、NeuN和oligo-2;Ki-67阳性率40%～60%。结论 s-PNET临床和影像学表现多样,确诊需要脑组织病理诊断。     

Immunohistochemistry of primitive neuroectodermal tumors in infants with special emphasis on cytokeratin expression

Summary Eleven primitive neuroectodermal tumor (PNET) biopsies from infants under the age of 3 years were studied for the presence of various differentiation markers for neuroectodermal stem cells. Special emphasis was placed on the expression of cytokeratin proteins. The tumor cells expressed different cytokeratin proteins (CK8, CK13, CK18, CK19, KL1, AE1/AE3, MNF16) in 3 of 11 cases. These cases were furthermore characterized by a strong expression of glial fibrillary acidic protein, S-100 protein and vimentin. Vimentin and cytokeratin proteins were co-expressed; cross-reactivity between these two intermediate filaments could be excluded by immunoblotting. It is noteworthy that the three positive tumors were all from infants in their 1st year. We assume that PNETs in early infancy are characterized by a particularly wide range of differentiation patterns. The presence of cytokeratin proteins in these cases seems to be associated with the expression of vimentin and must be regarded as an indicator of an early developmental stage of the tumor cells.     

Intra-axial endophytic primitive neuroectodermal tumors in the pons: clinical,radiological, and immunohistochemical aspects in four children

We present the clinical findings, radiological aspects, operative results, and histopathological features of four typical primitive neuroectodermal tumors (PNET) located in the pontine region in children. All the tumors had an endophytic intra-axial growth pattern. All the children had a short history of severe neurological deficits with involvement of the cranial nerves and pyramidal tract. MRI did not reveal any common feature of malignancy. Compared to our successful experience in operations of intra-axial endophytic brainstem tumors in a total of 32 children, the outcome was poor: all 4 children died within 13 months. We conclude that PNET occurring in the pons is not as rare as was believed, and, compared to PNET in other areas the prognosis is worse.     

椎管内原始神经外胚层肿瘤的诊断与治疗

目的 总结椎管内原始神经外胚层肿瘤的临床特点和治疗方法.方法 回顾性分析2005年1月至2010年12月上海华山医院治疗8例椎管内原始神经外胚层肿瘤的临床资料,对临床特点、诊断和治疗进行分析、总结.结果 8例患者均行显微外科治疗,其中全切除5例,次全切除1例,大部切除1例,部分切除1例.随访1-24个月,其中6例死亡,2例存活,平均生存时间12个月.结论 椎管内原始神经外胚层肿瘤发病率低,恶性度高,预后极差,常规病理辅以免疫组化和分子遗传学方法可明确诊断.手术切除肿瘤结合放化疗的综合治疗是目前的主要治疗原则.     

幕上原始神经外胚层肿瘤的临床、MRI及病理学特征

目的探讨幕上原始神经外胚层肿瘤(PNET)的临床、MRI及病理学特征。方法分析21例PNET患者的临床表现,术前进行头颅磁共振(MRI)扩散加权成像(DWI)检查,并测量肿瘤实质和瘤周水肿带的表观弥散系数(ADC)值,术后对脑肿瘤标本进行常规及免疫组化染色,分析ADC值与病理变化的关系。结果本组患者发病年龄在20岁以下11例(52%);临床表现以头痛、头晕、呕吐(16例)及视力异常(5例)为主,可伴嗅觉丧失或癫发作(3例)。MRI显示21例PNET均为单发病灶;病变位于幕上各脑区,其中额、颞、顶叶多发(18例);可跨脑区生长。病灶T1WI呈等信号或等低信号15例、散在高信号6例;T2WI呈等高混杂信号伴囊变坏死,4例瘤周水肿较轻,5例见血管流空征;增强后瘤体不均匀强化,4例见脑膜尾征样强化。14例DWI呈等高信号,囊变坏死区呈低信号。病理学显示肿瘤细胞形态主要向神经元(10例)和神经胶质细胞分化(8例),ADC值在不同分化类型的肿瘤间差异无统计学意义。免疫组化示神经元特异性烯醇化酶(NSE)、突触素(Syn)、胶质纤维酸性蛋白(GFAP)阳性率较多见。结论本组幕上PNET患者以20岁以下偏多,临床上以颅内压...     

A continuous cell line (KK-2) from a supratentorial primitive neuroectodermal tumor

Summary Tumor tissue located in the occipital lobe with hemorrhage was obtained from a 19-year-old patient. Histological examination indicated it to consist of undifferentiated small, round cells without neuronal or glial differentiation, and possibly to be a type of primitive neuroectodermal tumor. The tumor cells were cultured for 3 years and a continuous cell line (KK-2) was established. KK-2 was transplantable to nude mice. With immunocytochemistry, neuron-specific enolase, protein gene product 9.5, vimentin, TUJ1 (a monoclonal antibody specific for neuron-associated class III -tubulin isotype) and 6H7 (a monoclonal antibody to NCAM produced by us) were detected. None of the following could be found: glial fibrillary acidic protein, S-100 protein, neurofilament and synaptophysin, calcitonin gene-related peptide, gastrin releasing peptide corticotropin-releasing factor, substance P, somatostatin, chromogranin, aromatic l-amino acid decarboxylase and tyrosine hydroxylase. The original tumor and KK-2 cells obtained after 3 years of culture and transplants in nude mice displayed essentially the same ultrastructural and immunohistochemical characteristics. KK-2 cells showed no differentiation to mature neuronal, glial or ependymal cells. This cell line may possibly serve as a useful model for studying cellular differentiation of human neuroectodermal tumors and normal neuronal development.Supported in part by a Grant-in-Aid for Cancer Research from the Ministry of Health and Welfare (1–5), Japan     

Adipose tissue and smooth muscle in a primitive neuroectodermal tumor of cerebrum

We describe a case of an occipital lobe primitive neuroectodermal tumor (PNET) in a 60-year-old man with immunocytochemical evidence of neuronal, glial, smooth muscle and adipose tissue differentiation in the primary tumor and its metastases. This is the first case of PNET of cerebrum in which adipose tissue and smooth muscle has been demonstrated. The possible cells of origin for these mesenchymal elements is discussed.