颅咽管瘤的分型与显微外科治疗进展

显微外科技术的发展、影像学的进步、激素替代疗法的完善,使颅咽管瘤的疗效有了很大提高,但其远期预后、对并发症和死亡率的控制仍不令人满意,尤其在肿瘤的切除原则、手术入路的选择、术后放疗与否、垂体柄的保留等问题上存在着较大分歧。1 颅咽管瘤的外科学分型 根据肿瘤的位置和生长方式,Rougerie于1962年首先提出了颅咽管瘤的外科学分型,将颅咽管瘤分为5型:鞍内型、鞍内-鞍上向前扩展型、视交叉后型、巨大型、不典型型。1980年,Ciric将颅咽管瘤分为脑室内型、软脑膜下型、软脑膜外蛛网膜下型、蛛网膜内外型(哑铃型)、蛛网膜外型(单纯鞍内型)。1983年,Konovalov将颅咽管瘤分为鞍内型、鞍上     

颅咽管瘤侵袭第三脑室的方式对手术切除的影响

目的 探讨大型颅咽管瘤与第三脑室的关系在肿瘤切除手术中的意义. 方法 南方医科大学南方医院神经外科白1997年1月至2003年1月共采用手术治疗大型颅咽管瘤患者72例.根据肿瘤的影像学表现及手术所见对其进行分类,每一类肿瘤根据大小及其与第三腩室底的关系分为3级,根据术中判断和术后CT和(或)MPd增强扫描结果确定颅咽管瘤手术切除程度,分析肿瘤分级与手术切除程度的关系. 结果颅咽管瘤总体上可以分为第三脑室内型(本组7例)和第三脑室累及型(本组65例)两大类;手术切除程度在不同分级肿瘤间总体分布位置不同,差异有统计学意义(P<0.05). 结论 明确大型颅咽管瘤与第三脑室底及下丘腩结构间的关系对提高大型颅咽管瘤手术疗效有重要意义.     

颅咽管瘤MRI表现与术后水钠代谢紊乱关系分析

目的分析MRI上颅咽管瘤与第三脑室底的关系及对估计术后水钠代谢紊乱的意义.方法回顾性分析64例颅咽管瘤,根据术前MRI上颅咽管瘤与第三脑室底的关系将其分为4级,术后钠代谢情况分为正常、低钠、高钠及混合4组,尿量分为正常、轻度尿崩及严重尿崩3组,分别进行统计学分析.结果各级颅咽管瘤与钠代谢情况、尿量之间均有统计学差异.结论颅咽管瘤对第三脑室底侵袭越多,术后水钠代谢紊乱程度越重.分析MRI上颅咽管瘤与第三脑室底的关系,可以在一定程度上预测术后部分下丘脑反应的严重程度.     

颅咽管瘤手术前后的内分泌变化

颅咽管瘤是多发生于鞍上区并常累及下丘脑的良性肿瘤。颅咽管瘤的发病率国内尚无系统报道,北美报告0.13/10万,占北美人颅内肿瘤的4.7%～7.9%,在澳大利亚占颅内肿瘤的1.5%,在日本占颅内肿瘤的3.9%犤1犦;国内报道儿童颅咽管瘤约占儿童颅内肿瘤的5%,成人为3.5%犤2,3犦。颅咽管瘤生长于下丘脑的垂体柄、漏斗和正中隆起部位,向上生长可进入第三脑室,堵塞脑室系统,病人主要以头痛为主要表现;当肿瘤在鞍上生长时,肿瘤将第三脑室底抬起并向上压迫,垂体柄和漏斗向前或侧方移位,视交叉和视神经被压向前下方,病人主要表现为视力障碍、多饮、多尿和发育…     

颅咽管瘤血管内皮生长因子表达的研究

目的　探讨血管内皮生长因子 (VEGF)在颅咽管瘤中是否表达、肿瘤血管生成和囊肿形成中是否发挥作用。方法　根据术前影像和术中所见将 31例颅咽管瘤大体形态进行分类 ,标本行病理学检查和分型 ,用LSAB免疫组化方法 ,Ⅷ因子相关抗原单克隆抗体染色显示微血管并进行微血管计数 ,VEGF多克隆抗体染色显示VEGF并行阳性结果判断 ,将VEGF表达情况与肿瘤大体形态、病理类型、微血管计数进行统计学分析。结果　 31例中 9例肿瘤细胞有VEGF表达 ,VEGF表达率为 2 9% ,鳞状表皮型 13例中 ,7例有VEGF表达 ,牙釉质表皮型 18例仅 2例有VEGF表达 ,第Ⅷ因子染色见于所有肿瘤基质结缔组织内的微血管内皮细胞。VEGF表达与鳞状表皮型或实质性肿瘤呈显著正相关 (P <0 0 5 )。结论　鳞状表皮型颅咽管瘤VEGF表达明显高于牙釉质表皮型 ,提示VEGF可能在鳞状表皮型肿瘤的生物学行为中发挥重要作用     

QST分型对儿童颅咽管瘤术后血钠水平异常的影响

目的探讨不同QST分型颅咽管瘤患儿术后血钠水平异常的发生特点。方法回顾分析2016年1月至2017年6月经外科手术治疗的48例原发性颅咽管瘤患儿的临床、影像学及手术资料,分析不同QST分型对患儿术后血钠水平的影响及差异性。结果(56.25%),中至重度12例次占25%;高钠血症32例次(66.67%),中至重度16例次占33.33%。不同分型组患儿低钠血症(χ~2=11.420,P=0.003)和高钠血症(χ~2=15.702,P=0.000)发生率差异具有统计学意义,其中T型颅咽管瘤患儿术后中至重度低钠血症(Z=-3.324,P=0.001)和中至重度高钠血症(Z=-3.874,P=0.000)发生率高于Q型;单因素Logistic回归分析显示,QST分型是术后发生中至重度血钠水平异常的重要影响因素,T型为其危险因素(OR=315.422,95%CI:16.804～5920.786;P=0.000)。结论颅咽管瘤患儿术后应进行血钠水平长期监测,QST分型有助于预测血钠水平异常的发生及其严重程度,T型颅咽管瘤患儿可能为术后血钠水平异常的易感者。     

手术切除颅咽管瘤十年体会(附309例报告)

目的 总结分析10年来手术切除颅咽管瘤的经验.方法 回顾分析从1996年1月起10年间手术治疗309例颅咽管瘤患者.男162(52.4%)例,女147(47.6%)例.年龄在15岁以上者259(83.8%)例,平均年龄为35.8岁,15岁以下者占50例(16.2%),平均年龄8.8岁.肿瘤大小为2.0～9.0cm,平均3.4cm.肿瘤分成第三脑室底下型284(91.9%)例和第三脑室底上型25(8.1%)例.第三脑室底下型肿瘤,翼点入路209(67.6%)例,额底裂经终板入路55(17.8%)例,额下入路20(6.5%)例.第三脑室底上型肿瘤,经终板入路14(4.5%)例,经胼胝体入路11(3.6%)例.结果 肿瘤全切除276(89.3%)例,近全切除20(6.5%)例,大部切除13(4.2%)例.垂体柄保留186(60.2%)例,切断49(15.9%)例,不能鉴别74(23.9%)例,围手术期死亡12(3.9%)例.204(66%)例患者有6个月至8年的随访,平均2.1年,5例患者在随访中死亡,4例与下丘脑垂体功能低下有关.结论 术前通过神经影像学评价颅咽管瘤与第三脑室底、垂体柄和视交叉结构的关系,以第三脑室底作为标志,将肿瘤分为第三脑室底上型和下型选择手术入路可获得较好的肿瘤切除及较好的下丘脑结构和穿通动脉的保护,并可有效降低手术后并发症.术后一些患者神经内分泌功能的障碍仍是难题,尚需要多种激素的补充或代替治疗.     

第三脑室颅咽管瘤的手术治疗

目的探索手术切除第三脑室颅咽管瘤的适应证、方法、并发症预防及预后。方法对我科2000年1月至2004年4月期间收治并采取经皮质-侧脑室入路切除的38例第三脑室颅咽管瘤，结合患者术前症状和体征、影像学特点、手术效果、随访情况等进行回顾性分析。结果38例颅咽管瘤采用经皮质-侧脑室入路切除肿瘤，其中3例联合翼点入路，手术死亡率为0，达到术中全切的为28例（73．7％）。术后癫痫发作和术区硬脑膜下积液分别为2例（5．3％）和4例（10．5％），患者术后综合效果良好。平均随访26．3个月，7例（18．9％）患者复发。结论经侧脑室入路能安全切除部分第三脑室颅咽管瘤，死亡率、致残率低，术后无严重并发症，手术效果好。     

经终板入路显微外科手术切除视交叉后颅咽管瘤

目的 探讨经终板入路显微外科手术切除视交叉后累及第三脑室颅咽管瘤的可行性、手术技巧及疗效.方法 回顾性分析34例位于视交叉后累及第三脑室颅咽管瘤病人的临床资料,均采用经终板入路显微手术切除肿瘤.结果 肿瘤全切除30例,次全切除4例.本组死亡1例.术后出现尿崩症、电解质紊乱、垂体前叶功能低下等并发症.术前症状多数不同程度好转.23例随访3～7年,次全切除的病人中术后行伽玛刀治疗3例,随访期间未复发;拒绝放疗1例,术后16个月MRI示肿瘤复发.另19例肿瘤全切除病人随访期间均未复发.结论 经终板入路切除视交叉后累及第三脑室的颅咽管瘤,可取得较好的疗效;精湛的显微外科技巧可避免或减少发生术后并发症.     

颅咽管瘤钙化与骨化关系的研究

目的 通过对颅咽管瘤超微结构的研究,病理学及影像学资料的复习,探讨颅咽管瘤骨化特点及其形成机制. 方法 收集南方医科大学南方医院神经外科自2004年5月至2006年3月手术的颅咽管瘤标本54例,其中男31例;女23例.术后病理证实为成釉细胞型颅咽管瘤41例,鳞状细胞型颅咽管瘤13例.分析肿瘤标本的显微结构、组织类型、骨化的特征.透射电镜分析颅咽管瘤骨化的超微结构基础,结合手术及影像学资料,分析颅咽管瘤骨化及其与钙化的关系. 结果 成釉细胞型颅咽管瘤钙化情况:7例(-),11例(+),11例(++),12例(++++);鳞状细胞型颅咽管瘤钙化情况:13例(-).镜下可见蛋壳样钙化及大块钙化的颅咽管瘤存在骨化,而在鳞状细胞型颅咽管瘤、无钙化及砂粒样钙化的成釉细胞型颅咽管瘤标本中未见骨化的现象;骨化结构的分布与钙化的程度密切相关. 结论 颅咽管瘤骨化的发生与钙化程度存在密切的联系,钙化是颅咽管瘤骨化的基础,骨化的形成使原本没有生物学活性的钙化结构变成含有肿瘤细胞、肿瘤间质的复合体,为术后残留的钙化组织导致肿瘤复发提供了基础.     

鞍隔孔区解剖学研究及其临床意义

目的 通过鞍隔孔区结构的解剖学研究,分析经蝶入路脑脊液漏的发生机制,以及空蝶鞍(ES)的形成原因. 方法 对8例胎儿标本进行组织学连续切片后,做HE和Masson染色,并在显微镜下对鞍隔孔附近结构进行观察;另取10例成人尸头标本,模拟经蝶入路手术,并在手术显微镜下观察鞍隔孔区解剖结构. 结果 鞍上蛛网膜在垂体柄上端和其表面的软脑膜紧密结合,并转折进入鞍内;同时在垂体上表面处,鞍隔从四周紧密包绕并和表面的软脑膜紧密结合,而难以从组织学切片上分辨二者的界限:鞍上蛛网膜池由于蛛网膜、软脑膜和鞍隔的束缚而终止于鞍隔孔上部. 结论 鞍隔、软脑膜和鞍上蛛网膜三者之间存在着严密的解剖学关系,其也是防止脑脊液漏和ES发生的关键因素.这三者的先天性缺损、生理性或者病理性破坏,尤其是在经蝶入路中对垂体腺瘤的过分牵拉,导致鞍上蛛网膜和软脑膜分离或者破裂,可能是造成术中脑脊液漏发生的重要原因;另外鞍隔、软脑膜和垂体上表面之间的分离也可能是造成ES的关键因素.     

Meninges play a neurotrophic role in the regeneration of vasopressin nerves after hypophysectomy

Following hypophysectomy the regenerating fibers of magnocellular neurons are known to establish new neurohemal connections with reorganized vasculatures in the median eminence, which lead to establishment of a posterior pituitary-like structure. In order to examine the role of the meninges (the pia mater and the arachnoid) in this regeneration process, we implanted the meningeal tissues obtained from neonatal rat pups into the third ventricle of the adult rats, and then hypophysectomized the host animals. Ten days after hypophysectomy, the meningeal tissue grafts were found to be densely innervated by regenerating vasopressin-immunoreactive fibers. Such fibers had dots and frequently formed large punctuations. On the contrary, few vasopressin fibers were found within the cortical tissue grafts. Further, the exposure of primary hypothalamic cell cultures to the medium conditioned by meningeal cell cultures promoted not only the survival of vasopressin-immunoreactive neurons but also the outgrowth and arborization of the neurites. The survivals of cortical and cerebellum neurons in culture were also promoted by the conditioned medium. These findings raise the possibility that the meninges play an important role in the axonal regeneration process after hypophysectomy.     

CD34 and dural fibroblasts: the relationship to solitary fibrous tumor and meningioma

Intracranial solitary fibrous tumors (SFTs) are typically dural-based, CD34-positive neoplasms of uncertain histogenesis. We examined ten cases of meninges obtained at autopsy from patients with no history of neurological illness, head trauma, or neurosurgical intervention, and ten cases of typical meningiomas with attached dural margins not involved by tumor. All cases were immunostained with CD34. CD34 reactivity was noted in the long, thin delicate processes of dural fibroblasts preferentially located in the meningeal portion of the dura rather than the periosteal portion. No CD34 reactivity was identified in the arachnoid or pia mater, except in some endothelial cells. One supratentorial dural-based fibrous nodule and one SFT within the confines of the fourth ventricle showed strong and diffuse reactivity to CD34, bcl-2, and vimentin, and were negative for epithelial membrane antigen (EMA), S-100 protein, glial fibrillary acidic protein, smooth muscle actin, and desmin. We also describe a meningothelial meningioma within which a well circumscribed SFT-like nodule was embedded. The SFT-like nodule was strongly CD34 positive and EMA negative, and the meningioma was strongly EMA positive and CD34 negative. Fibroblasts of the dural border cell layer are attached to the underlying arachnoid, and their inclusion with arachnoidal stromal elements and pial-based tela choroidea during formation of choroid plexus interstitium may account for intraventricular SFTs. Our results suggest that SFTs and dural-based fibrous nodules derive from CD34-positive dural-based fibroblasts, and that CD34 reactivity in meningiomas may result from inclusion of dural fibroblasts within the neoplasm.     

脑膜癌病的脑脊液细胞学诊断相关因素研究

目的 探讨影响脑膜癌病脑脊液细胞学诊断的相关因素。方法 回顾性分析46例脑膜癌病患者的临床资料。结果 46例脑膜癌病的脑脊液中，38例发现瘤细胞(约82．6％)；40例腰穿脑脊液中，35例检到瘤细胞；6例脑室穿刺脑脊液中，3例检到瘤细胞；35例腰穿脑脊液检到瘤细胞中，26例为第一次检到瘤细胞，5例第2次检到瘤细胞，3例第3次检到瘤细胞，l例3次以上检到瘤细胞；6例硬脑膜-蛛网膜强化者仅l例脑脊液细胞学检到瘤细胞；4例软脑膜-蛛网膜强化者脑脊液细胞学均检到瘤细胞；38例原发肿瘤中，19例血液系统肿瘤(白血病、淋巴瘤)均检到瘤细胞，7例肺癌检出4例，5例胃癌检出3例，4例乳腺癌检出2例，1例室管膜瘤全部检出，1例脑淋巴瘤全部检出。结论常规脑脊液细胞学检查具有较高的特异性，脑脊液的来源、腰穿检查的次数、脑膜癌病病变的位置、原发肿瘤及组织类型均为影响其敏感性的重要因素。     

The intracranial arachnoid mater

Introduction

The arachnoid mater is a delicate and avascular layer that lies in direct contact with the dura and is separated from the pia mater by the cerebrospinal fluid-filled subarachnoid space. The subarachnoid space is divided into cisterns named according to surrounding brain structures.

Methods

The medical literature on this meningeal layer was reviewed in regard to historical aspects, etymology, embryology, histology, and anatomy with special emphasis on the arachnoid cisterns. Cerebrospinal fluid dynamics are discussed along with a section devoted to arachnoid cysts.

Conclusion

Knowledge on the arachnoid mater and cerebrospinal fluid dynamics has evolved over time and is of great significance to the neurosurgeon in clinical practice.     

大鼠实验性脑室出血后LN及LNR-1在脑内的表达及意义

目的观察大鼠脑室出血后层粘蛋白（LN）及其受体LNR-1在大鼠脑内的表达变化，探讨其与出血后慢性脑积水之间的关系。方法将大鼠随机分为3组：正常对照组、假手术对照组、实验组，将枸橼酸化的0．1ml自体静脉血注入实验组大鼠侧脑室内建立出血后慢性脑积水模型（假手术组注入0．1ml生理盐水），采用免疫组化方法观察出血后不同时间LN及LNR-1在脑内的表达变化。结果实验组30d出现脑积水为80％（4／5）、其余组别未出现慢性脑积水；实验组出血后LN在纵裂池、软脑膜、蛛网膜、硬膜、脑内毛细血管壁、蛛网膜下腔血管壁的表达在3d时增加，7d表达继续增加，14d表达最强，较对照组、假手术组显著增强（P〈0．01），30d仍强烈表达；实验组出血后LNR-1在脉络丛的立方上皮细胞胞浆及顶质膜、室管膜细胞、软脑膜细胞、蛛网膜细胞、硬膜、纵裂池、胶质细胞的表达增加。LNR-1在蛛网膜下腔的表达变化与LN基本一致。结论大鼠脑室出血后LN及LNR-1的表达上调可能参与蛛网膜纤维化的过程，从而在出血后慢性脑积水的形成中发挥重要作用。     

The Cranial Arachnoid and Pia Mater in Man: Anatomical and Ultrastructural Observations

The objects of the present study were: (1) to define the relationships of the arachnoid mater to blood vessels in the subarachnoid space; (2) to establish the structure of leptomeningeal trabeculae and their relationships to the pia mater; and (3) to investigate the fine structure of the human pia mater. Intracranial portions of vertebral artery were taken at post mortem, and normal cerebral cortex and overlying leptomeninges were obtained from surgical lobectomies. Tissue from these specimens was examined by scanning and transmission electron microscopy, by light microscopy and by immunocytochemistry for the presence of basement membrane, desmosomal proteins and vimentin. Results of the study showed that as the vertebral artery pierced the posterior atlanto-occipital membrane and entered the subarachnoid space, it acquired a leptomeningeal coat as the arachnoid was reflected on to it. It has been demonstrated previously that as vessels enter the brain, the leptomeningeal coat is reflected on to the surface of the cortex as the pia mater. The arachnoid mater was seen to consist of a subdural mesothelial layer and a compact central layer as previously reported. From the inner layer of the arachnoid, collagen bundles coated by leptomeningeal cells extended as trabeculae across the subarachnoid space to fuse with the pia mater. The pia itself was composed of a delicate but apparently continuous layer of cells joined by desmosomes and gap junctions but no tight junctions were observed. It was possible to detect pia mater cells in the perivascular spaces of the brain by immunocytochemical techniques using light microscopy. The significance of the observed anatomical arrangement for cerebrospinal fluid physiology is discussed.     

Ganglioside in Meningiomen und Hirnhäuten

Summary The ganglioside concentrations and the ganglioside patterns were investigated in 18 meningiomas of different histological type and in the meninges. The ganglioside concentration in the meningiomas ranged from 0.16 to 1.63 mg ganglioside N-acetylneuraminic acid per gram dry weight (mean 0.68 mg/g). The gangliosides G_M3, G_D3 and G_D1a were found to be the major ganglioside fractions of the meningiomas. In the endotheliomatous meningiomas, on teh average, the percentage of G_M3 was higher, and the percentage of G_D3 lower than in the endotheliomatous-fibromatous tumours. However, none of the individual ganglioside patterns observed seemed to be specific of a certain histological type.The meninges contained less gangliosides than the meningiomas. The arachnoideal ganglioside content was 0.17 mg/g, whereas the mean ganglioside concentrations in the dura mater and in the leptomeninx (probably representative of the pia mater) were as low as 0.07 and 0.08 mg/g, respectively. G_M3 represented the largest ganglioside fraction in the arachnoidea, and G_D3 in the dura mater. A high percentage of tetrahexosyl gangliosides was observed in the leptomeninx, but no, or very little G_D3.Of all the meninges, the results obtained with the meningiomas showed the closest resemblance to the arachnoideal findings. This observation is in agreement with current opinion that meningiomas probably originate from the arachnoidea.

Herrn Professor Dr. O. Hoffmann-Ostenhof zum 60. Geburtstag gewidmet     

Pial and arachnoid welding for restoration of normal cord anatomy after excision of intramedullary spinal cord tumors.

A significant postoperative problem in patients undergoing excision of intramedullary tumors is painful dysesthesiae, attributed to various causes, including edema, arachnoid scarring and cord tethering. The authors describe a technique of welding the pia and arachnoid after the excision of intramedullary spinal cord tumors used in seven cases. Using a fine bipolar forcep and a low current, the pial edges of the myelotomy were brought together and welded under saline irrigation. A similar method was used for closing the arachnoid while the dura was closed with a running 5-0 vicryl suture. Closing the pia and arachnoid restores normal cord anatomy after tumor excision and may reduce the incidence of postoperative painful dysesthesiae.