桥小脑角区不典型脉络丛乳头状瘤2例病理及免疫组化分析

目的 探讨桥小脑角不典型脉络丛乳头状瘤的临床特点、病理诊断及鉴别诊断.方法 对2例不典型脉络丛乳头状瘤的影像学、组织病理学和免疫组化特征进行研究,结合文献讨论其临床病理特征及鉴别诊断.结果 本文病例均病理形态多样,部分区域分化差,细胞密度增加、核分裂3～5个/10 HPF,呈片状方式生长.免疫组化结果:CK、V imentin、S-100呈阳性,Syn呈弱阳性；其他标记物如EMA、GFAP、CEA均呈阴性,Ki-67阳性率局部＞5％.结论 患者年龄、肿瘤准确定位等临床资料对病理诊断较为重要；严格掌握肿瘤的诊断标准并辅以合理的免疫组化检查,对诊断与鉴别诊断极为重要.     

右侧额叶转移性沙砾体型肺乳头状腺癌

目的报告1例临床罕见的右侧额叶转移性沙砾体型肺乳头状腺癌患者,结合文献探讨其临床病理学特点、诊断与鉴别诊断要点。方法与结果男性患者,56岁,临床表现为额枕部间歇性头痛。MRI显示右侧额叶占位性病变,考虑脑膜瘤可能。术中可见右侧额叶肿瘤呈灰红色、质地较韧、边界清楚、血供丰富。组织学形态观察,肿瘤组织呈乳头状结构,胞质嗜酸性,胞核呈圆形或卵圆形,可见小的红核仁,肿瘤组织和肿瘤间质中可见大量沙砾体形成;免疫组织化学染色,肿瘤细胞弥漫性表达细胞角蛋白(CK)、上皮膜抗原、CK19、甲状腺转录因子-1和Napsin A蛋白,灶性表达P53,Ki-67抗原标记指数为5%~10%;不表达胶质纤维酸性蛋白、S-100蛋白、甲状腺球蛋白、Galectin-3蛋白、孕激素受体、波形蛋白等标志物。结合胸部18F-FDG-PET CT显像,最终病理诊断为(右侧额叶)转移性沙砾体型肺乳头状腺癌。术后辅以放射治疗和药物化疗,随访12个月,肿瘤无复发。结论中枢神经系统转移性沙砾体型肺乳头状腺癌临床罕见,应详细了解病史,并注意与乳头状型脑膜瘤、脉络丛乳头状瘤、乳头状型室管膜瘤、乳头状型胶质神经元肿瘤和转移性甲状腺乳头状癌等相鉴别,以免误诊或漏诊。     

异位脉络丛癌(附1例报告及文献复习)

目的介绍脉络丛乳头状癌的病理和免疫组化特征。方法对一例脉络丛乳头状癌患者的临床表现、组织形态学和免疫组化染色进行分析,并结合文献对本病的诊断、鉴别要点进行了探讨。结果脉络丛乳头状癌的组织学形态特点:由柱状上皮围绕毛细血管、纤维组织所形成的乳头样结构。瘤细胞有异型,可见核分裂相。免疫组化示细胞角蛋白(CK)阳性,胶质纤维酸性蛋白(GFAP)、S-100及波形蛋白(Vimentin)灶性阳性,上皮膜抗原(EMA)、突触素(Syn)、神经元特异性烯醇化酶(NSE)、神经微丝(NF)及均为阴性。Ki67:>20%。PAS染色阴性。结论脉络丛乳头状癌的诊断主要依靠其组织学形态和免疫组化标记;常需与室管膜瘤、转移癌及乳头瘤型脑膜瘤鉴别。     

脉络丛乳头状瘤影像学诊断及临床治疗研究

目的探讨脉络丛乳头状瘤的CT、MRI表现及临床治疗方法。方法回顾性分析我院收治的28例经术后病理证实为脉络丛乳头状瘤（包括1例不典型者）患者的临床资料。本组病例术前均行CT、MRI检查。结果肿瘤位于侧脑室者19例,位于第四脑室者7例,位于第三脑室者2例;所有患者均行显微手术治疗,肿瘤完全切除22例,部分切除6例（术后均行放射治疗）。术后5个月至5年,随访平均3．2年,21例治愈,4例复发,2例死亡,1例失访。结论MRI常呈颗粒状混杂信号且浸泡于脑脊液中为脉络丛乳头状瘤典型的影像学表现;手术切除是目前其首选治疗措施;放射治疗是未全切除者术后的主要辅助治疗手段。     

室管膜下巨细胞星形细胞瘤的临床及病理特点分析(附2例报告)

目的探讨室管膜下巨细胞星形细胞瘤(SEGAs)的临床、病理特征和免疫组化标记及鉴别诊断。方法分析2例SEGAs患者的临床特点,观察组织病理切片和免疫组化标记。结果 2例SEGAs患者的发病年龄均小于20岁,肿瘤位于侧脑室壁,由肥胖型肿瘤细胞/节细胞样巨细胞和长梭形肿瘤细胞组成,均伴钙化。免疫组化显示,肿瘤细胞表达GFAP、S-100、vimentin、Map-2、Nestin、SOX-2、Caspase-3、Caspase-9、Bax,不表达Neun、CD133、CD34、Ki-67。结论 SEGAs常伴结节硬化复合症,可能起源于多潜能的神经干细胞,存在凋亡机制的激活;需与肥胖细胞型星形细胞瘤、室管膜瘤、脉络丛乳头状瘤、室管膜下瘤进行鉴别。     

《中国微侵袭神经外科杂志》2008年第13卷主题词索引

A阿片相关性障碍阿片相关性障碍的人格特征及伏隔核毁损手术对其的影响(附26例分析)(吕博川,李智勇,杜鹏,等)(5):204B不典型脉络丛乳头状瘤不典型脉络丛乳头状瘤1例:临床及病理研究(陈晓东,王伟民,张伟,等)(5):207C垂体肿瘤Letin、PTTG、Ki67的表达与垂体腺瘤侵袭性的研究(李军     

脉络丛癌(附5例报告)

目的　探讨脉络丛癌的诊断和治疗。方法　分析我院诊治的 5例脉络丛癌临床资料。结果　临床表现为进行性颅高压征 ,影像学表现不典型 ;免疫组化特异性不高 ;随访均未见复发。结论　脉络丛癌在成年人临床诊断困难 ,治疗以手术为主 ,力争肿瘤全切除     

105例脑肿瘤卒中的临床诊治和病理基础

目的 总结脑肿瘤卒中的临床、病理特点，提高临床诊治水平。方法 对手术及病理证实的脑肿瘤卒中105例的临床资料进行回顾性分析。结果 垂体腺瘤49例，星形细胞瘤18例，转移瘤11例，少突胶质细胞瘤7例，多形性胶质母细胞瘤5例，髓母细胞瘤4例，脑膜瘤2例，桥小脑角神经鞘瘤2例，生殖细胞瘤2例，黑色素瘤2例，恶性淋巴瘤1例，室管膜瘤1例，脉络丛乳头状瘤1例。肿瘤卒中表现为颅内血肿、蛛网膜下腔出血或硬膜下血肿。结论 肿瘤的血管性变化是脑肿瘤卒中的摹础，CT扫描对于早期诊断和治疗有重要意义，脑肿瘤卒中易误诊，需与其他出血性疾病加以鉴别。     

显微手术治疗低龄儿童幕上脉络丛肿瘤

目的总结6岁以下低龄儿童幕上脉络丛肿瘤的临床特点和手术经验。方法回顾性分析2004年1月至2010年12月显微手术治疗的13例6岁以下儿童脉络丛肿瘤患者的临床资料。本组13例,平均年龄为19．4月;肿瘤位于侧脑室7例、第三脑室内6例。结果肿瘤全切除10例,次全切除2例,部分切除1例。术后病理证实12例为脉络丛乳头状瘤,l例为脉络丛乳头状癌。术后随访3～92个月,平均38．1月;1例脉络丛乳头状癌患者术后6月复发并转移而死亡;1例脉络丛乳头状瘤患者术后16个月死于颅内感染;余11例存活。结论儿童幕上脉络丛肿瘤大部分为良性肿瘤,显微手术全切除肿瘤可达到满意的疗效。     

脊索瘤样脑膜瘤1例报道及文献复习

目的 探讨脊索瘤样脑膜瘤的诊断、治疗。方法 回顾性分析1例右侧颅内蝶骨嵴起源的脊索瘤样脑膜瘤的临床资料,并结合文献进行分析。结果 MRI和CT与一般类型的脑膜瘤一样具有典型脑膜尾征,但是CT密度（低密度）和MRI信号（长T1、长T2信号）特点不同于常见类型。手术全切肿瘤,术后12个月颅脑MRI复查显示肿瘤未复发。结论 脊索瘤样脑膜瘤是具有脊索瘤样肿瘤特点的脑膜瘤,其影像特征及病理基础均不同于一般类型的脑膜瘤,要注意与其他脊索瘤样肿瘤相鉴别。手术全切肿瘤是主要治疗方法,但是易复发,注意长期随访。     

Rapid regrowth of an atypical choroid plexus papilloma located in the cerebellopontine angle

Choroid plexus papilloma usually occurs in the lateral or the fourth ventricle. Primary choroid plexus papilloma of the cerebellopontine angle, as described here, is an uncommon lesion. A 42-year-old man presented with a 1-month history of dysphagia and gait unsteadiness. CT scans and MRI showed a large extra-axial tumor in the right cerebellopontine angle. Pathological study revealed that the lesion was choroid plexus papilloma. Repeat imaging conducted 1 year after the operation showed that the tumor had recurred with distinct cystic features. Pathological examination again revealed increased mitotic activity and supported a diagnosis of atypical choroid plexus papilloma.     

Atypical Choroid Plexus Papilloma in an Adult

We present an extremely rare case of the atypical choroid plexus papilloma in an adult which developed at the trigone of right lateral ventricle. A 62-year-old woman presented with the history of intermittent and gradually progressive headache and left side hemiparesis for 6 months. The brain magnetic resonance image showed highly enhanced and well demarcated mass at the trigone of lateral ventricle attached to the choroid plexus. Gross total resection was performed by transcortical approach via the middle temporal gyrus. The tumor was diagnosed as an atypical choroid plexus papilloma. She had no neurologic deficit after the surgery. We report a case of atypical choroid plexus papilloma in adult and introduce newly classified pathologic characteristics of this tumor.     

Clinicopathologic correlations in epithelial choroid plexus neoplasms: a study of 52 cases

Summary Sixty-seven tumor specimens of epithelial choroid plexus neoplasms obtained by 60 biopsies and 7 autopsies from 52 patients were investigated. Diagnoses of the first operations were choroid plexus papilloma (PP; 32 cases), choroid plexus papilloma with histological atypies (atypical PP; 6 cases), and choroid plexus carcinoma (PC; 14 cases). Carcinoembryonic antigen was expressed by 2 of the 3 biopsies autoptically recognized as metastatic carcinomas and by 2 autopsy cases of PC, while it was absent in all biopsies of true choroid plexus tumors. Tumor cells positive for transthyretin (TTR, prealbumin), S-100 protein (S100), and glial fibrillary acidic protein (GFAP) were detected in 39, 46 and 13, respectively, of the 49 cases of true choroid plexus tumors. Fourth ventricle tumors expressed more S100 (number of positive tumor cells) than lateral ventricle tumors, PP more S100 and TTR than atypical PP/PC. Tumors from patients 20 years of age and older expressed more GFAP and TTR than tumors from younger patients. Of the 30 patients with complete follow-up 19 were alive 2 to 11 years after surgery, including 7 recurrencies. Eleven died from the tumor 4 months to 7 years after surgery. The following histopathologic features (in order of decreasing significance) were correlated with poor prognosis (recurrency or fatal outcome): less than 50% of the tumor cells heavily positive for S100, presence of mitoses, absence of TTR-positive cells, brain invasion by cell nests, absence of marked stromal edema, and presence of necrotic areas. Our results indicate that some histologic features correlate significantly with poor prognosis and that immunohistochemical results correlate with tumor localization, age, and malignancy.     

Differential expression of cell adhesion molecules (CAM), neural CAM and epithelial cadherin in ependymomas and choroid plexus tumors

A series of frozen specimens of 18 ependymomas and 7 choroid plexus tumors were examined for their expression of cell adhesion molecules, such as neural cell adhesion molecule (NCAM), its polysialylated isoforms (PSA NCAM), and epithelial (E-) cadherin, and of intermediate filament proteins, such as glial fibrillary acidic protein (GFAP) and cytokeratin, using various monoclonal and polyclonal antibodies. Normal choroid plexus and ependyma were taken as controls. Anti-E-cadherin immunoreactivity was observed on the basolateral part of most adult choroid plexus and benign choroid plexus papilloma cells. However, a small number of atypical papillomas and carcinoma cells showed anti- E-cadherin immunoreactivity throughout their cell surface membrane. NCAM were not expressed by adult choroid plexus and benign papilloma cells. Only a few cells expressed NCAM and PSA NCAM in developing choroid plexus, atypical papillomas and carcinomas. Cytokeratin expression was always observed in choroid plexus and their tumors; GFAP expression was variable from case to case. In contrast, ependymal cells and their tumors never expressed E-cadherin but strongly expressed NCAM. PSA NCAM was found in ependymomas exhibiting anaplastic features. All ependymomas strongly expressed GFAP and a few demonstrated slight expression of cytokeratin. These data suggest that, besides GFAP and cytokeratin, NCAM and E-cadherin are of potential diagnostic value in distinguishing choroid plexus tumors from ependymomas. E-cadherin and NCAM may play a role in the functional organization of normal choroid plexus and ependyma, respectively. In particular, incomplete or irregular anti-E-cadherin expression in choroid plexus tumors and PSA NCAM immunoreativity in ependymomas and choroid plexus tumors correlates with the emergence of anaplastic histological features.     

Clinicopathologic study of endolymphatic sac tumor (ELST) and differential diagnosis of papillary tumors located at the cerebellopontine angle

We investigated the clinicopathologic features and immunophenotypes of 10 cases of endolymphatic sac tumor (ELST) and compared them with other papillary tumors, including eight cases of choroid plexus papilloma (CPP), three cases of atypical choroid plexus papilloma (ACPP), two cases of papillary ependymoma (PE), three cases of papillary meningioma (PM) and two cases of metastatic carcinoma (MC) the at cerebellopontine angle (CPA). The age at onset of ELST ranged from 13 to 39 years. The male‐to‐female ratio was 1:1. The clinical presentations were primarily ear‐related symptoms. The temporal bones showed extensive destruction. Histologically, the important characteristics for differential diagnosis with CPP, which is most similar to ELST, include the quantity of blood vessels, the nuclei location at apical surface of the papillary, clear cytoplasm cells sometimes with visible vacuoles, psammoma bodies and dura or bone invasion. Immunohistochemistry stains for AE1/AE3, cytokeratin CK)5/6, epithelial membrane antigen, CK8/18, S‐100, and synaptophysin are helpful in diagnosis of ELST. In ELST, ultrastructure of uniform 2 μm vesicles in cytoplasm was seen, and gene analysis also showed missense mutation in exon 3. This study indicates that the above histological features combined with immunohistochemistry findings are important for making the correct diagnosis. Gene analysis should be used in patients without medical history to exclude von Hippel‐Lindau disease.     

Primary choroid plexus papilloma of the cerebellopontine angle presenting as brain stem tumor in child.

A case of a primary choroid plexus papilloma of the cerebellopontine angle in an 8-year-old female is presented. The clinical features of progressive cranial nerve palsies and cerebellar signs in the absence of intracranial hypertension initially suggested an intrinsic brain stem lesion. Whereas a radionuclide brain scan demonstrated abnormal uptake in the region of the pons, vertebral angiography and pneumoencephalography were diagnostic of an angle mass. At surgery, the tumor proved to be a choroid plexus papilloma that was totally confined to the cerebellopontine angle. This patient represents the thirteenth reported case of a primary cerebellopontine angle papilloma and the first such case occurring in a child.     

黏液乳头型室管膜瘤9例临床病理分析

目的探讨黏液乳头型室管膜瘤的临床病理特征、鉴别诊断及治疗与预后。方法回顾性分析9例黏液乳头型室管膜瘤病人的临床资料,行组织学观察及免疫组织化学分析。结果 9例肿瘤均发生于脊髓圆锥、马尾终丝区,典型病理形态为立方形或梭形肿瘤细胞,以乳头放射状结构排列在血管黏液样间质轴心周围,大量黏液聚集在血管和瘤细胞之间或微囊内,伴血管壁增厚、玻璃样变;瘤细胞大小、形态较一致,核分裂像少见或无。免疫组织化学显示肿瘤细胞胶质纤维酸性蛋白(GFAP)、vimentin及S-100阳性,细胞角蛋白(CK)及上皮膜抗原(EMA)阴性。病人均手术全切,术后辅助放疗2例。6例随访17个月～5年,MRI复查仅1例复发。结论黏液乳头型室管膜瘤特征性发病部位、形态学特征及免疫表型是其诊断和鉴别诊断要点,肿瘤全切后预后良好。     

Immunocytochemical detection of insulin-like growth factor II (IGF-II) in choroid plexus papilloma: a possible marker for differential diagnosis

BACKGROUND: Insulin-like growth factor II (IGF-II) has been detected in the choroid plexus of animals by means of immunohistochemistry and in situ hybridization, and this factor is thought to play an important role in the central nervous system (CNS). Little is known, however, about the presence and localization of this substance in the choroid plexus and in choroid plexus papilloma in humans. MATERIALS AND METHODS: 5 normal choroid plexus and 10 choroid plexus papillomas were examined for IGF-II by immunocytochemistry. IGF-II was not detected in normal human choroid plexus, whereas it was found in choroid plexus papilloma. Furthermore, to assess the possibility that IGF-II could serve as an immunohistological marker of choroid plexus papilloma, we used the same technique to examine paraffin-embedded samples from various kinds of brain tumors. RESULTS AND CONCLUSION: Our results suggest that IGF-II may be a useful marker for choroid plexus papilloma in differential diagnosis.     

鞍区软骨样脊索瘤简

目的探讨鞍区软骨样脊索瘤的临床病理学特征,复习相关文献。方法与结果男性患者,66岁。临床表现为反复性低钠血症,MRI显示鞍区类圆形、边界清楚占位性病变。经右侧鼻腔入路行内镜下鞍区肿瘤切除术,术中可见肿瘤呈实性,质地坚韧,边界清楚;组织形态学表现为典型的脊索瘤组成,肿瘤组织内呈灶性软骨样分化;肿瘤细胞表达细胞角蛋白、上皮膜抗原和S-100蛋白,Ki-67抗原标记指数约为1％,病理诊断为鞍区软骨样脊索瘤。术后随访11个月,一般状况良好,肿瘤无复发。结论软骨样脊索瘤多生长于中轴骨骼,具有特殊的组织学构象和免疫表型,患者预后较典型脊索瘤好,诊断时应注意与典型脊索瘤和软骨肉瘤相鉴别。