小脑发育不良性节细胞瘤/Lhermitte-Duclos病6例临床病理分析并文献复习

目的探讨小脑发育不良性节细胞瘤/Lhermitte-Duclos病(Lhermitte-Duclos disease,LDD)的临床病理学特征、诊断及鉴别诊断。方法对6例LDD进行临床病理学分析,行免疫组化SP法染色,并结合文献对该病的临床表现、组织学形态、免疫表型及预后进行分析。结果 6例患者年龄23~56岁,平均34岁,临床症状表现为颅内压增高伴或不伴小脑体征等。MRI表现为特征性"虎斑征"。光镜下表现为局部小脑结构紊乱,颗粒细胞及浦肯野细胞减少并代以多量异常神经节细胞。随访2例5~8年复发,其余4例恢复良好。结论 LDD属于小脑的罕见原发性良性病变,诊断依靠影像学和病理组织学检查,治疗方法首选完整切除。     

小脑节细胞瘤1例报道

小脑发育不良性节细胞瘤(Lhemitte-Duclos disease,LDD)十分少见,为中枢神经系统少见的良性肿瘤,病理学上认为它可能属迷芽瘤或错构瘤性质[1].此病多发于青少年期,可能合并的发育畸形有巨脑畸形、小脑回畸形、脑灰质异位、脊髓积水和多指畸形等.     

肝脏原发粒层细胞瘤伴海绵状血管瘤1例报道并文献复习

目的探讨肝脏原发粒层细胞瘤(granulosa cell tumor)伴海绵状血管瘤的临床病理学特点。方法对1例肝脏原发粒层细胞瘤伴海绵状血管瘤进行组织学观察和免疫组化染色,观察其超微结构及临床影像学特点,并复习相关文献。结果患者女性,CT及MRI提示肝脏右叶可见一巨大实性肿物,低密度。肉眼见肿物突向肝脏下方,切面呈实性;另外,在肝脏膈面被膜下见一灰红色出血区,呈疏松海绵状。镜下巨大肿物中肿瘤细胞椭圆形或梭形,呈微滤泡状、小梁状排列,部分区域呈实性弥漫状分布,类似于卵巢粒层细胞;部分区域呈束状排列,间质中可见较多胶原纤维瘢痕形成,似孤立性纤维性肿瘤。肿瘤细胞核内可见小核仁,并可见纵形核沟。免疫组化结果示肿瘤细胞CD99、α-inhibin、CK8、CK18、SMA和BCL-2均(+),Ki-67增殖指数<1%。超微结构显示肿瘤细胞可见各种形式的核皱叠形成,部分细胞呈Call-Exner小体样结构排列,偶见桥粒结构,提示该肿瘤与卵巢粒层细胞瘤极其相似。结论粒层细胞瘤原发于肝脏且合并海绵状血管瘤,非常罕见,目前其临床生物学行为难以确定,需对患者进行长期随访。     

Primary granulosa cell tumor of liver associated with cavernous hemangioma: a case of report and review of literature

目的 探讨肝脏原发粒层细胞瘤(granulosa cell tumor)伴海绵状血管瘤的临床病理学特点.方法 对1例肝脏原发粒层细胞瘤伴海绵状血管瘤进行组织学观察和免疫组化染色,观察其超微结构及临床影像学特点,并复习相关文献.结果 患者女性,CT及MRI提示肝脏右叶可见一巨大实性肿物,低密度.肉眼见肿物突向肝脏下方,切面呈实性;另外,在肝脏膈面被膜下见一灰红色出血区,呈疏松海绵状.镜下巨大肿物中肿瘤细胞椭圆形或梭形,呈微滤泡状、小梁状排列,部分区域呈实性弥漫状分布,类似于卵巢粒层细胞;部分区域呈束状排列,间质中可见较多胶原纤维瘢痕形成,似孤立性纤维性肿瘤.肿瘤细胞核内可见小核仁,并可见纵形核沟.免疫组化结果示肿瘤细胞CD99、α-inhibin、CK8、CK18、SMA和BCL-2均(+),Ki-67增殖指数＜1%.超微结构显示肿瘤细胞可见各种形式的核皱叠形成,部分细胞呈Call-Exner小体样结构排列,偶见桥粒结构,提示该肿瘤与卵巢粒层细胞瘤极其相似.结论 粒层细胞瘤原发于肝脏且合并海绵状血管瘤,非常罕见,目前其临床生物学行为难以确定,需对患者进行长期随访.     

关注神经元和混合性神经元-胶质肿瘤的临床病理研究

神经元和混合性神经元-胶质肿瘤（neuronal and mixed neuronal-glial tumours）是一组有不同程度神经元分化和胶质分化的肿瘤,近些年来该类肿瘤得到了越来越多的重视。在最新出皈的WHO中枢神经系统肿瘤分类（2007版,简称WHO2007年分类）中,变化最大的就属神经元和混合性神经元胶质肿瘤这一章节。在WHO2007年分类中共新增了八个新的肿瘤类型,而有关神经元和混合性神经元胶质肿瘤这一章节中就增加了三种新肿瘤,使其包括了12种肿瘤类型;小脑发育不良性神经节细胞瘤（Lhermitte-Duelos）、婴儿促纤维增生性星形细胞瘤／节细胞胶质瘤（desmoplastic infantile astrocytoma／ganglioglioma）、胚胎发育不良性神经上皮肿瘤（dysembryoplastic neuroepithelial tumour,DNT）,     

卵巢幼年性粒层细胞瘤伴Maffucci综合征

目的 探讨幼年性粒层细胞瘤和Maffucci综合征的临床病理特征及两者伴发的原因。方法 对1例伴Maffucci综合征的幼年性粒层细胞瘤进行HE、组织化学及免疫组织化学染色观察，并复习文献。结果 幼年性粒层细胞瘤多发生于20岁以下患者，儿童患者常表现为青春期前假性性早熟，生育期妇女常表现为月经紊乱。组织学上具特征性滤泡结构，丰富的嗜酸性或空泡化胞质，无核沟及Call—Exner小体，核分裂象多见。Inhibin免疫组化染色有助于诊断和鉴别诊断。Maffucci综合征为先天性软骨发育异常，易合并恶性肿瘤。14例卵巢幼年性粒层细胞瘤伴Maffucci综合征或Ollier病中卵巢肿瘤和骨病变有同侧分布倾向。结论 幼年性粒层细胞瘤合并Maffucci综合征可能为系统性中胚叶发育异常所致。     

基质结构蛋白在脑血管畸形中的表达差异

背景：有研究表明脑血管畸形中存在管壁结构基质蛋白的表达差异,但血管畸形间结构基质蛋白的差异及结构蛋白差异与畸形血管管壁异形关系少有报道。 目的：观察基质结构蛋白在脑血管畸形中的差异性表达。 方法：对50例脑血管畸形手术标本和34例正常颞浅动脉标本进行苏木精-伊红染色,并应用美国Santa Cruz公司生产单/多克隆抗体对4种基质结构蛋白-Ⅳ型胶原蛋白、a-血管平滑肌蛋白、层粘连蛋白和纤维连接蛋白免疫组织化学染色。 结果与结论：苏木精-伊红染色显示,脑血管畸形壁结构与正常血管相比呈现明显紊乱和构成异形性;且脑血管畸形中,脑动静脉畸形与海绵状血管瘤结构存在明显差异。免疫组织化学染色显示：正常颞浅动脉和畸形血管中Ⅳ型胶原和a-血管平滑肌蛋白全部呈阳性表达且无显著性差异,但结构排列差异明显;正常颞浅动脉与畸形血管层粘连蛋白和纤维连接蛋白表达阳性率差异有显著性意义(P < 0.05),正常血管表达更多层粘连蛋白,畸形血管表达更多纤维连接蛋白;畸形血管中脑动静脉畸形和海绵状血管瘤间层粘连蛋白和纤维连接蛋白的阳性率表达差异有显著性意义(P < 0.05),脑动静脉畸形血管表达更多层粘连蛋白,海绵状血管瘤血管表达更多纤维连接蛋白。结果证实：与正常血管相比,脑血管畸形者管壁结构存在明显异形性,结构蛋白Ⅳ型胶原和a-血管平滑肌蛋白结构明显紊乱,且低表达层粘连蛋白而高表达纤维连接蛋白,这种差异可能是导致脑血管壁结构异常的原因。 中国组织工程研究杂志出版内容重点：组织构建;骨细胞;软骨细胞;细胞培养;成纤维细胞;血管内皮细胞;骨质疏松;组织工程全文链接：     

脑膜血管周细胞瘤2例报道并文献复习

目的 探讨颅内血管周细胞瘤(haemangiopericytomas,HPC)的发病率、临床病理特点、遗传学改变.方法 对2例颅内HPC进行光镜、电镜、免疫组化分析并复习颅外HPC1例、血管瘤型脑膜瘤2例与孤立性纤维性肿瘤2例的临床病理资料.结果 2例均由密集的梭形细胞组成,其内含丰富的鹿角状血管,网状纤维包绕单个瘤细胞,免疫组化CD34+、CD99+、Bcl-2+.结论 颅内HPC是一种不同于脑膜瘤与孤立性纤维性肿瘤的少见肿瘤.     

血管瘤样纤维组织细胞瘤1例报道并文献复习

血管瘤样恶性纤维组织细胞瘤(angiomatoid malignant fibroos histocytona,AMFH)是一种少见的软组织肿瘤,也有文献称之为血管瘤样纤维组织细胞瘤.WHO(1994)将AMFH确认为中间型(低度恶性)纤维组织细胞肿瘤,其临床及病理学特点不同于普通类型恶性纤维组织细胞瘤,易误诊.笔者报道1例AMFH的光镜及免疫组化观察结果,并结合文献讨论其临床病理特征及诊断与鉴别诊断.     

神经垂体少见肿瘤15例临床病理分析

以光镜、电镜、组化和免疫组化等方法，研究了１５例发生于神经垂体（包括漏斗突、漏斗柄和正中隆起）的少见肿瘤：星形细胞瘤１２例（其中３例伴垂体腺瘤、１例伴灶性节细胞增生）；节细胞瘤２例（１例伴侵袭性垂体腺瘤）；颗粒细胞瘤１例。临床均有闭经泌乳等内分泌症状和视力改变及内分泌检验异常等。因瘤体多较小，且质地稀软，临床、手术、放射和病理检查常被忽视。手术疗效佳，术后短期内症状体征消失或明显改善。此外，还探讨了节细胞瘤和颗粒细胞瘤的起源，讨论了临床、手术、放射和病理检查等应注意的问题等。     

5-Hydroxytryptamine and Dopamine Neurons in the Cerebellum of the New-Hatching Yangtze Alligator Alligator sinensis

Nissl and immunohistochemical staining methods were used to morphologically characterize the cerebellum of the new-hatching Yangtze alligator, and the cerebellar histological structure and the distribution profiles of 5-hydroxytryptamine (5-HT) and dopamine (DA) neurons were investigated for the first time. The results of cerebellar histological structure showed that there was a ventriculus cerebelli in the cerebellum of the new-hatching Yangtze alligator, the surface of the cerebellar cortex was not very smooth, the cerebellar cortex could be divided into four layers, which include external granular layer, molecular layer, Purkinje cell layer and granular layer, Purkinje cell layer could be characterized specially by multilayer, two cerebellar nuclei termed as the nucleus cerebelli lateralis and the nucleus cerebelli medialis were found in the cerebellar medulla. 5-hydroxytryptamine-immunoreactive (5-HT-IR) and dopamine-immunoreactive (DA-IR) neurons and fibers distributed widely in the cerebellum. The structures and profiles of 5-HT-IR and DA-IR neurons and fibers in the cerebellum of the Yangtze alligator were similar to that reported in other reptiles, but also had some specific features. The abundance of 5-HT and DA in cerebellum suggested that these highly conserved neurotransmitters would play important roles in motor control. Anat Rec, 302:861–868, 2019. © 2018 Wiley Periodicals, Inc.     

猫小脑皮质结构的年龄相关性变化

目的：对青年和老年猫小脑皮质结构的年龄性变化进行比较。方法：动用Nissl染色显示小脑皮质神经元,免疫组织化学方法显示胶质纤维酸性蛋白免疫阳性（GFAP-IR）星形胶质细胞和神经丝蛋白免疫阳性（NF-IR）结构。显微镜下观察测量小脑皮质厚度和细胞密度。结果：与青年猫比较,老年猫小脑皮质总厚度及分子层厚度显著下降,颗粒层厚度明显增加,各层神经元密度明显降低;颗粒层中GFAP-IR细胞密度显著增加,阳性反应增强;老年猫蒲肯野细胞（PC）NF免疫阳性树突分支明显减少。结论：衰老过程中小脑皮质神经元丢失和PC中NF阳性树突减少,可能会导致老年小脑皮质接受和整合信息的功能降低,而星形胶质细胞活动增强对皮质神经元可能起保护作用。     

Endothelium in hepatic cavernous hemangiomas does not express the hyaluronan receptor for endocytosis

The liver contains two distinct endothelial cell types: vascular and sinusoidal. Although cavernous hemangioma is the most common benign tumor of the liver, vascular or sinusoidal endothelial cell differentiation has not been described. An endocytic receptor responsible for the uptake and degradation of hyaluronan is present in the sinusoidal endothelium of the liver. The hyaluronan receptor for endocytosis (HARE) may therefore be a useful marker for sinusoidal endothelial cell differentiation. Using monoclonal antibodies specific for HARE, CD31, and factor VIII, we completed an immunohistochemical study of the endothelial cells of both hepatic cavernous hemangiomas and of nonneoplastic human liver. The anti-HARE monoclonal antibodies showed diffuse strong staining of nonneoplastic liver sinusoidal endothelium. No staining of nonsinusoidal endothelium or the endothelial lining of the hemangiomas was seen with anti-HARE. In contrast, diffuse strong staining for factor VIII and CD31 was present in nonsinusoidal endothelium and cavernous hemangioma endothelium. Neither factor VIII nor CD31 staining was present in the sinusoidal endothelium. In conclusion, the endothelium of hepatic cavernous hemangiomas demonstrates vascular but not sinusoidal differentiation based on the absence of HARE and presence of CD31 and factor VIII.     

Immunohistochemical demonstration of glutamate dehydrogenase in the postnatally developing rat hippocampal formation and cerebellar cortex: comparison to activity staining.

Distribution patterns of activity and immunohistochemical staining for glutamate dehydrogenase were compared during the postnatal development of rat hippocampal formation and cerebellar cortex. On postnatal day 5, dendritic layers of the hippocampal formation showed only a very weak enzyme activity. Similarly, when studied at the same age, the external granule cell layer and Purkinje cells of the cerebellar cortex exhibited a very faint and moderate staining, respectively. With advancing age, in both brain regions a marked postnatal increase in glutamate dehydrogenase activity occurred in neuropil area as glutamatergic structures matured. However, compared to activity staining, both brain regions of early postnatal stages showed a relatively high level of glutamate dehydrogenase-like immunoreactivity. In this case, the immunohistochemical staining of hippocampal dendritic layers and of the molecular layer of the cerebellar cortex was rather diffuse, being not very similar to parameters of the maturation of the respective glutamatergic structures. In contrast to the activity staining for the enzyme, the immunohistochemical labelling in adult rats revealed a selective predominance of immunoreactivity in astroglial cells from postnatal day 5 onwards. The Bergmann glia in the cerebellar cortex exhibited the strongest intensity of immunoreactivity. Generally, the patterns of immunoreactivity were found to depend on the fixation procedure adopted. Concluding from our results, glutamate dehydrogenase is demonstrable in glial and in neuronal cell elements as well. Therefore, it is recommended that activity staining and the immunohistochemical procedure be combined to study qualitative and quantitative aspects of glutamate dehydrogenase in nervous tissues.     

A developmentally regulated axonal glycoprotein (7-8D2 antigen) with a restricted distribution in mature rat brain

7-8D2 is a mouse monoclonal IgGl antibody which recognizes a neuronal cell surface antigen in rat brain. Immunohistochemical techniques reveal the antigen to be present most abundantly in the cerebellum of the adult brain, where it is expressed by the cell bodies and fibres of the granule neurons. Lower levels of staining were found in the neuropil of the hippocampus, in some fibres of the corpus callosum and in the most superficial layer of the cerebral cortex. Immunoelectron microscopy confirmed that the antigen was present on the surface of the parallel fibres in the cerebellum and showed that it was absent from glial, Purkinje or stellate cell membranes. The antigen had a more widespread distribution early in development, and the restricted adult distribution was achieved by the end of the second postnatal week. Immunoblotting of samples of adult rat brain shows that the antigen appears as a close doublet of bands at 211,000 mol. wt. This result was confirmed by immunoprecipitating the antigen from metabolically labelled glycoproteins prepared from cultured cerebellar interneurons. Immunoblotting and immunohistochemical experiments were in agreement that the cerebellum contained high levels of the antigen, and that lower but significant amounts could be found in other brain regions, notably the hippocampus and the cerebral cortex. The localization data and the changes in the distribution of the antigen may suggest some role for this molecule during early brain development.     

Immunoreactivity for calretinin and other calcium-binding proteins in cerebellum

Two calcium-binding proteins, calbindin and parvalbumin, have been reported to be abundant in Purkinje cells and other cell types in the cerebellum. Immunoreactivity for a related protein, calretinin, is now reported in cerebellum of chick and rat. In the chick, antibodies against calretinin stain mossy fibres throughout, and climbing fibres in a distinct group of folia. They also stain several cell types in the molecular layer. As there is no detectable calretinin mRNA in the cerebellar cortex, this cellular staining may be due to cross-reaction with an unknown antigen. In the rat, antibodies against calretinin stain the Lugaro cells, and some granule cells in lobe X; they also give weak staining of all the granule cells in the other lobes. Thus almost all the neuronal cell types in the cerebellum show immunoreactivity for at least one of the calcium-binding proteins in one or both species.     

Architecture of Purkinje cells of the reeler mutant mouse observed by immunohistochemistry for the inositol 1,4,5-trisphosphate receptor protein P400.

P400 protein, which is identical to the inositol 1,4,5-trisphosphate receptor protein, is a glycoprotein closely associated with the membranes of Purkinje cells. Three types of monoclonal antibodies against P400 protein were employed for the immunohistochemical detection of Purkinje cells in the cerebellum and brainstem of the normal and reeler mouse. Purkinje cells in both types of mice were immunoreactive against anti-P400 antibodies, and the soma, dendrites, axon and even terminal boutons in the cerebellar and vestibular nuclei could be clearly visualized. In the cerebellum of the reeler mutant, the heterotopic Purkinje cells both within and below the granule cell layer were also immunopositive and could be clearly differentiated from the deep cerebellar nuclei, in which neurons were immunonegative. The molecular layer of the reeler cerebellum varied in thickness and certain parts were completely defective. The dendrites within the molecular layer extended from Purkinje cells whose cell bodies were located in the normal position, abnormally in the granule cell layer, or at the surface of the central mass. Outside the cortex of the cerebellum, ectopic Purkinje cells were demonstrated in 3 cerebellar nuclei, the cerebellar medulla and peduncle, and brainstem of the normal and reeler mouse.     

Localization of 5-HT2A, 5-HT3, 5-HT5A and 5-HT7 receptor-like immunoreactivity in the rat cerebellum

Although serotonin (5-hydroxytryptamine, 5-HT) is known to exert a modulatory action on cerebellar function, our current knowledge of the nature of receptor subtypes mediating serotonergic activity in this part of the brain remains fragmentary. In this study, we report the presence and distribution of 5-HT3, 5-HT5A and 5-HT7 receptor-like immunoreactivity in the rat cerebellum using immunofluorescence histochemistry. 5-HT3 immunoreactivity was found in fibers sparsely distributed throughout the cerebellum. Most of them were seen in the cerebellar cortex as fine varicose 5-HT3-positive axonal processes. 5-HT5A immunoreactivity, on the other hand, was observed in neuronal somata of the cerebellar cortex and deep cerebellar nuclei. Based upon cell morphology and the use of cell-specific markers, Purkinje cells, molecular layer interneurons and Golgi cells were found to be 5-HT5A immunopositive. In addition, the use of cell-specific markers allowed us to identify previously reported large 5-HT2A-positive cells in the granular layer as being Golgi cells. Finally, 5-HT7 immunoreactivity was observed only in Purkinje cells. Corroborating previous radioligand-binding, in situ hybridization and immunohistochemical studies, our data relate serotonin receptor subtypes to specific cerebellar cell types and may consequently contribute to the elucidation of serotonergic actions in the cerebellum.     

Retroperitoneal kaposiform hemangioendothelioma with tufted angioma-like features in an infant with Kasabach-Merritt syndrome

Kasabach-Merritt syndrome denotes profound thrombocytopenia and coagulopathy in an infant with a vascular tumor. A retroperitoneal vascular tumor with an unusual combination of histopathological features is reported, and compared with vascular lesions described in the reported cases of Kasabach-Merritt syndrome in the literature. A large retroperitoneal tumor that had expanded through the sigmoid mesocolon into the sigmoid colon wall was resected from an 8-month-old infant with fully developed Kasabach-Merritt syndrome. Histological examination revealed a combination of venous (cavernous) malformation, kaposiform hemangioendothelioma and tufted angioma-like areas. Cellular tumor components (especially tufted angioma-like parts) infiltrated the wall of the sigmoid colon to the submucosal level. Immunohistochemical staining with antibodies to the Ki-67 antigen and proliferating cell nuclear antigen showed a low proliferative activity, whereas the antiapoptotic bcl-2 protein was expressed diffusely in tumor cells. This is the first reported case of a vascular tumor with tufted angioma-like elements found in the retroperitoneum, and the first reported in combination with kaposiform hemangioendothelioma and venous malformation in the same lesion. Considering the immunohistochemical results and overlapping histological features, it may be considered that tufted angioma and kaposiform hemangioendothelioma represent different growth patterns or stages in the development of a single type of hemangioma.     

小鼠小脑皮质发育中的神经元凋亡

目的探讨小鼠小脑皮质发育中神经元凋亡的规律和机制。方法用激活型Caspase-3多抗免疫组织化学标记及Hoechst 33258染色液染色,检测从出生至成年小鼠小脑皮质中神经元的凋亡,用Western blotting方法对小脑组织中Caspase-3和Caspase-8的活化片段进行半定量测定。结果外颗粒层、普肯耶细胞层和内颗粒层凋亡细胞密度最高分别在出生后第8d(P8)、P5及P9,P20各层凋亡细胞密度都很低。Caspase-3活化片段的表达量在P5较高,P5以后逐渐降低,至P14消失;Caspase-8活化片段的表达量从P0到P10都较高,P10以后逐渐降低,至P30消失。结论P0至P14是小脑皮质神经元凋亡的重要时期,通过启动Caspase-8的活化进而激活效应性Caspase-3的细胞凋亡途径存在于小脑皮质的塑型成熟过程。