基于QST分型的颅咽管瘤与第三脑室底脑膜层次的关系

目的探索基于QST分型的、累及第三脑室底的颅咽管瘤与第三脑室底脑膜层次的关系及临床意义。方法回顾性分析2018年1月至2019年10月南方医科大学南方医院神经外科在神经内镜下行肿瘤全切除术治疗累及第三脑室底的原发性颅咽管瘤患者的临床资料(共17例,其中Q型6例,S型3例,T型8例),所有患者术中均留取肿瘤组织标本。正常鞍区标本来源于同期该院行人工引产或自然流产的胎儿(8例)。对以上标本进行苏木素-伊红(HE)和免疫荧光双标染色,应用波形蛋白抗体标记硬脑膜,Ⅰ型胶原蛋白抗体标记蛛网膜,胶质纤维酸性蛋白抗体和层粘连蛋白抗体标记软脑膜,以CK18抗体标记腺垂体,以CK5/6抗体标记颅咽管瘤。观察胎儿脑组织标本的脑膜染色情况以及不同QST分型的颅咽管瘤组织与第三脑室底脑膜的层次关系。结果8例胎儿标本均成功标记硬脑膜、蛛网膜、软脑膜。颅咽管瘤标本HE染色及免疫荧光双标染色结果显示,所有Q型肿瘤(6/6)与第三脑室底之间存在硬脑膜(鞍膈);所有S型肿瘤(3/3)与第三脑室底之间存在蛛网膜和软脑膜;T型肿瘤与第三脑室底之间存在3种病理学形态关系,分别命名为卯榫样、地幔样及护城河样关系,所有T型肿瘤(8/8)与第三脑室底之间有软脑膜分隔,但在肿瘤起源点处,软脑膜可逐渐消失。当肿瘤极度挤压第三脑室空间时,第三脑室室管膜仍可保持完整。结论不同QST分型的颅咽管瘤均可累及第三脑室底,且与第三脑室底之间存在不同的脑膜层次;这些脑膜层次是手术安全切除累及第三脑室底的颅咽管瘤的天然屏障。     

神经内镜手术治疗脑室内肿瘤

目的探讨神经内镜手术治疗脑室内肿瘤的临床疗效及经验.方法回顾性分析16例经神经内镜手术治疗的脑室内肿瘤的临床资料,其中肿瘤单纯位于侧脑室8例,第三脑室6例,同时位于侧脑室和第三脑室2例.结果肿瘤全切除4例,部分切除8例,囊性颅咽管瘤经囊腔内置Ommaya管术后囊腔明显缩小4例.术后根据病理检查对部分病例进行放射治疗.均无明显手术并发症.随访2～36个月,平均15.7个月,无死亡病例,第三脑室内生殖细胞瘤复发1例,第三脑室内囊性颅咽管瘤囊腔扩大1例.结论神经内镜手术治疗脑室内肿瘤具有微创、手术视野好、并发症少等优点,是一种有效的诊疗手段.     

Craniopharyngioma of the pineal region

Craniopharyngiomas generally develop either in the suprasellar region or in both suprasellar and intrasellar regions. We report on a nontypical location of the craniopharyngioma in the pineal region. An 8-year-old boy was admitted to the department of pediatric neurosurgery in a grave condition. An MRI scan of the brain was performed after the neurological examination and revealed a large neoplasm situated in the posterior part of the III ventricle and in the pineal region, measuring 8.5×6.5×5 cm. The size of the tumor and its location meant it was occluding three ventricles, with subsequent hydrocephaly. Total removal of the tumor was carried out. Microscopic investigation of the tissue removed showed the typical structure characteristic for craniopharyngioma of the adamantinomatous type. Results of a consultation 6 months after the operation revealed that the patient was feeling well, attending school regularly and had finished the first semester with excellent results. On control MRI scan no tumor was found. Received: 14 January 1998     

导航引导下内镜扩大经鼻蝶窦入路切除鞍上区和第三脑室内颅咽管瘤

目的 探讨导航引导下完全内镜扩大经鼻蝶窦入路切除位于鞍上区和第三脑室内颅咽管瘤的可行性和有效性.方法 采用内镜扩大经鼻蝶窦入路、术中导航引导下切除3例位于鞍上区和第三脑室内的颅咽管瘤.结果 3例颅咽管瘤全切,手术效果好.术后随访10-14个月,患者生活正常,需要激素替代治疗.结论 内镜扩大经鼻蝶窦入路可以安全有效地切除位于鞍上区、第三脑室内的颅咽管瘤,这种手术方式不需要牵拉脑组织,并能完全暴露视交叉后、下方区域,在直视下操作,有利于对下丘脑、垂体柄及其他重要结构的保护.对于选择性的颅咽管瘤病例,内镜扩大经鼻蝶窦入路是切除肿瘤的一种新型微创手术入路.神经导航可以验证解剖标记点,引导手术方向,增加手术安全性.

Abstract：

Objective To investigate the feasibility and efficacy of image -guided extended endoscopic endonasal transsphenoidal approach(EEETA) for the removal of craniopharyngiomas in the suprasellar region and third ventricle. Method A pure EEETA with image -guided system was used. Three patients with a craniopharyngioma involving the suprasellar region and third ventricle were treated. Results Total craniopharyngioma removal was achieved in three cases. All the patients recovered uneventfully. The follow - up study was carried out for 10 to 14 months with good outcomes. Compensatory endocrine substitution therapy was needed in all of them. Conclusions The EEETA for removal of craniopharyngiomas in the suprasellar region and third ventricle is feasible and effective. It has the advantages of no needing for brain retraction,offering panoramic view of retrochiasmatic and infrachiasmatic regions,manipulating under direct vision and protecting hypothalamus,pituitary stalk and other vital structures. The EEETA is a novel and minimally invasive approach for selected cases of craniopharyngioma. Neuronavigation plays an important role in identifying anatomic landmarks,guiding surgical direction and increasing safety of the operations.     

Pedicled craniopharyngioma of the 3d ventricle

A 62 year-old man suffered of headache and progressive walking difficulties for 4 years. Radiological examinations showed a calcified intraventricular tumor attached to the floor of the 3rd ventricle. Death, caused by septicemia, occurred before neurosurgery. On sagittal braincut the tumor appeared pediculated and was attached to the anterior part of the floor of the 3rd ventricle. The microscopic features were those of typical craniopharyngioma. The pedicle and the floor of the 3rd ventricule were devoid of tumoral cells. Numerous large vessels which originated in the basal leptomeninges were present in the ventricular floor and the pedicle and then branched out into the tumor. Pathologically proven purely intraventricular craniopharyngiomas have been seldom reported. To our knowledge an autopsy case of pediculated intraventricular craniopharyngioma has been previously described only once, without particular attention to the pedicle. The integrity of the floor of the 3rd ventricle constitutes the only feature that may differentiate with certainty an intraventricular extension of a suprasellar craniopharyngioma from a pure intraventricular form of this tumor.     

Craniopharyngioma in the third ventricle: necropsy findings and histogenesis.

A case of craniopharyngioma confined within the third ventricle with necropsy is reported. A stalk-like structure in this tumour was present in the wall of the third ventricle at its base. It is suggested that this tumour might have arisen from the remnants of Rathke's pouch persisting in the tuber cinereum.     

Severe amnesic syndrome and collecting behavior after surgery for craniopharyngioma.

Significant neuropsychologic sequelae were induced by total removal of craniopharyngioma via a frontobasal interhemispheric approach. A 50-year-old right-handed man developed severe amnesic syndrome and collecting behavior after total removal of a craniopharyngioma. He had very poor results on tests of learning, recall, and recognition for both verbal and nonverbal tasks. Magnetic resonance imaging revealed damage to the bilateral mammillary bodies and fornices, and N-isopropyl-p-[23I]iodoamphetamine single photon emission computed tomography showed decreased cerebral blood flow in the bilateral frontal lobes, predominantly in the right, and regions around the third ventricle. The present case suggests that damage to the brain structures surrounding the third ventricle associated with surgery for craniopharyngioma may result in amnesic syndrome and collecting behavior. Generally, the frontobasal interhemispheric approach is the optimum choice for the removal of craniopharyngioma without significant sequelae related to the surgical method, but the risk of neuropsychologic disturbances must be kept in mind.     

Cyst fenestration and Ommaya reservoir placement in endoscopic transcortical transventricular approach for recurrent suprasellar cystic craniopharyngioma without ventriculomegaly

The efficacy of the endoscopic transcortical transventricular approach (ETTA) for craniopharyngioma in the third ventricle with hydrocephalus has been reported focusing on its reduced invasiveness. On the other hand, suprasellar craniopharyngioma without ventriculomegaly is generally surgically managed by craniotomy or the endoscopic endonasal approach (EEA). Here, we report an elderly patient who received cyst fenestration and Ommaya reservoir placement in ETTA for recurrent suprasellar cystic craniopharyngioma without ventriculomegaly. The ETTA as a less invasive procedure is feasible in patients not only with intraventricular craniopharyngioma but also with suprasellar craniopharyngioma without hydrocephalus provided a navigational system is applied and the surgeon has ample experience with transcranial endoscopic procedures.     

A case of craniopharyngioma with chemical meningitis as an initial symptom]

We reported a rare case of craniopharyngioma with chemical meningitis due to spontaneous rupture of the tumor. A 50-year-old woman was admitted with high fever, headache, and nausea. On physical examination, she had nuchal rigidity. The examination of her cerebral spinal fluid(CSF) revealed pleocytosis(mononuclear cell dominant), low value of glucose level and high content of protein. The feature of her CSF findings suggested tuberculosis or fungal meningitis, but bacteriologic culture of the CSF was negative. The CT scan showed an isodensity mass in the suprasellar region and a spotty calcification in the third ventricle. The MRI with gadolinium enhancement suggested that the tumor must be craniopharyngioma and that meningitis was a type of chemical meningitis due to spontaneous rupture of craniopharyngioma. The corticosteroid therapy was rather effective to the symptoms of fever and headache. Then the operation was performed by neurosurgeons, and the diagnosis of craniopharyngioma was pathologically confirmed. Spontaneous rupture of craniopharyngioma rarely occurred and was followed by chemical meningitis. This case was an extremely rare condition that presented with chemical meningitis as an initial symptom.     

Removal of tumors in the III ventricle using the lamina terminalis approach

Tumors in the III ventricle were totally removed in three children using a route through the lamina terminalis. The cases are discussed on the basis of computed tomography and intraoperative findings. It seems that tumors 4×2 cm in size can be successfully removed via this relatively small opening if the neuroradiological findings and the probable histology (craniopharyngioma) provide secure evidence that the tumor site and growth matrix are located in the frontal and lower portion of the III ventricle. Besides the advantage of requiring no transparenchymal access, this quick axial (orthograde) approach exerts no pressure on the hypothalamus, a complication which cannot always be avoided with the transcallosal route or the route through the foramen of Monro. Furthermore, the immediate location of the tumor behind the usually protruding lamina terminalis permits a rapid operation without exploratory characteristics. The distance between the brain surface and the tumor with this procedure is 0 cm; however, it can be up to 9 cm, depending on the age of the patient, with other approaches.Presented at the 11th Meeting of the European Society for Paediatric Neurosurgery, Naples 1988     

儿童颅咽管瘤手术治疗和长期随访

目的探求在保护下丘脑功能的条件下，安全地伞切或近全切儿童颅咽管瘤。并长期随访术后病儿的生存情况。方法手术切除儿童颅咽管瘤202例。采用经胼胝体一透明隔间隙一穹隆问入路、经额部纵裂入路等八种入路切除鞍区和第三脑室内的颅咽管瘤。术前和术后采取积极措施预防和治疗下丘脑功能紊乱。并对其中的105例进行了12个月到6年的长期随访。结果全切＋近全切184例（91．1％），死亡2例（0．9％）。术后主要并发症为：尿崩症发生率80．7％，长期尿崩症率11．4％；血电解质紊乱74．8％；癫痫9．4％。术后第1年和第4年是死亡高峰。手术后5年生存率68．2％。结论选择合适的手术入路在直视下尽可能全切除颅咽管瘤，并保护第三脑室前下外侧壁（下丘脑）。术后积极防治血钠紊乱和癫痫是确保手术安全的重要措施。术后放疗可明显降低颅咽管瘤的复发率。     

Prolonged cerebral salt wasting following craniopharyngioma surgery and posterior reversible encephalopathy syndrome: a case report]

A 9-year-old boy was admitted to our hospital with daytime urinary incontinence for the past one year. MRI showed craniopharyngioma occupying the third ventricle. The tumor was excised by interhemispheric approach. Because hyponatremia and polyuria with high renal loss of sodium were observed on postoperative day 3, hydrocortisone and DDAVP were replaced. On postoperative day 24, successive general convulsions and hyponatremia recurred, and MRI FLAIR imaging showed marked brain edema in the bilateral parieto-occipital lobes. This finding disappeared late in the course of treatment, and the case was diagnosed as posterior reversible encephalopathy syndrome. The pathophysiology of cerebral salt wasting and posterior reversible encephalopathy syndrome in a craniopharyngioma patient are also discussed in the article.     

Intraventricular craniopharyngioma.

An unusual case of an adult with a craniopharyngioma within the third ventricle is reported. The patient complained of headaches, had a history suggestive of diabetes insipidus, and presented with a severe dementia. A brain scan revealed the suprasellar midline lesion, and a pneumoencephalogram confirmed its location within the third ventricle. Therapy included partial surgical excision, followed by a ventriculoatrial shunt and radiation.     

立体定向术治疗囊性颅咽管瘤--附170例报告

目的通过对立体定向术治疗囊性颅咽管瘤的回顾性分析,总结手术经验。方法应用Leksell-D/G型定向仪,本组170例患者中,24例仅行肿瘤囊腔穿刺冲洗术,15例单纯穿刺冲洗后注入8~16mg平阳霉素行囊腔内化疗,131例行肿瘤囊腔穿刺冲洗术及囊腔侧脑室分流术。结果169例患者术后症状缓解,术后随访135例中,42例复发,其中,28例为单纯穿刺冲洗及穿刺冲洗后注入平阳霉素,14例为定向分流术后,35例失随访。结论立体定向术治疗囊性颅咽管瘤可迅速缓解患者临床症状,对脑组织损伤小,术后并发症较少且较轻微,其中,定向分流术长期疗效优于单纯穿刺肿瘤囊腔冲洗及冲洗 平阳霉素内化疗。     

颅咽管瘤的显微手术治疗及随访

目的探讨翼点入路为基础的颅咽管瘤全切除方法，并通过长期随访了解患者生存状态。方法根据肿瘤影像学表现的生长部位及扩展方向将翼点入路进行改良，对121例颅咽管瘤患者进行手术治疗，并对其中83例进行12-84个月的随访。结果本组全切除、近全切率分别为79．3％、19．0％，术后最常见的肿瘤残留部位位于肿瘤与第三脑室底部前端连续处。术后最常见的下丘脑反应为尿崩症（86、8％）及低钠、高钠血症（68、6％），其中高钠血症预示严重的下丘脑反应，但经过处理术后反应多可良好控制。围手术期死亡3例（2．5％）。11例随访期内复发，复发率13％。随访期患者复发最常见于术后2年内。结论根据肿瘤生长方向及大小，选择各种改良翼点入路进行积极的全切除，多数颅咽管瘤可以得到良好的长期控制和生存质量。     

Recovery from anterograde and retrograde amnesia after percutaneous drainage of a cystic craniopharyngioma.

A case is reported of a cystic craniopharyngioma involving the floor and walls of the third ventricle. Pronounced anterograde and retrograde amnesia were documented preoperatively by formal testing. Rapid improvement in both new learning capacity and remote memory occurred after percutaneous twist drill drainage of the cystic portion of the tumour. The relevance of these observations to the amnesic syndrome and its neuropathological basis is discussed.     

颅咽管瘤非典型MRI表现

目的探讨颅咽管瘤非典型MRI表现,以提高对此病的认识。方法回顾性分析4例经手术病理证实的颅咽管瘤的MRI资料。结果4例肿瘤均为囊性:例1肿瘤位于鞍上,瘤内可见脂液平面,脑室、脑池及脑沟内可见大小不等的脂肪小滴;例2肿瘤的大部分位于桥小脑角区,仅小部分位于鞍上,T1WI和T2WI均为高信号,脂肪抑制亦为高信号;例3肿瘤位于鞍上,突向鞍内并延伸至胼胝体压部下方,蝶鞍稍增大,垂体未显示,T1WI和T2WI均为高信号;例4肿瘤位于鞍内及鞍上,蝶鞍扩大,垂体未显示,肿瘤前部T1WI和T2WI均为高信号,后部T1WI信号稍高,T2WI与白质信号相同。结论颅咽管瘤存在方式多种多样,有特征性的MRI表现,也可有非典型MRI表现,肿瘤可向邻近部位延伸,亦可破裂播散至蛛网膜下腔内。囊性颅咽管瘤的信号特点与囊液的成分有关。     

Craniopharyngioma and "reactive" subependymoma of the third ventricle--a case report

A 42-year-old woman with an 18-year history of suprasellar tumor, treated by repeated radiation therapy and surgery, was found to have both a craniopharyngioma and subependymoma of the third ventricle. The case is unique in that well documented subependymomas have not been reported in this location. Furthermore, only a few cases of subependymoma have been reported to be accompanied by a primary tumor of the central nervous system other than an ependymoma. Potential causative factors for this seemingly reactive subependymoma are briefly discussed.     

经基底纵裂间入路治疗鞍区肿瘤

目的 探讨经基底纵裂间入路治疗鞍区肿瘤的效果.方法 对15例鞍区肿瘤患者,采用经基底纵裂间入路,对手术效果进行分析总结.结果 15例患者肿瘤全切,1例颅咽管瘤患者因高渗性缺水,出院后死亡,2例颅咽管瘤患者纠正高渗性缺水后正常出院.1例颅咽管瘤患者术后半年有复发迹象,行γ-刀治疗,其余患者无复发,术后无颅内感染及脑脊液鼻漏发生,术后不影响美观.结论 经基底纵裂间入路,手术视野广泛,向上可以进入第三脑室,向后可显露中脑导水管,向下可进入鞍内,应用范围广,手术效果好.     

Recurrent sleep attacks associated with a craniopharyngioma

Symptomatic narcolepsy, once regarded as common, is now believed to be very rare. A 32-year-old man had a history of recurrent sleep attacks. A magnetic resonance imaging scan revealed a third ventricle tumor. The tumor was totally removed, and the histology was a craniopharyngioma. The symptoms ceased after the operation. The chronological correlation and the anatomical location of the tumor suggest that the patient had a symptomatic narcolepsy caused by the tumor. This is the first report that documents the cessation of narcolepsy attacks after tumor removal.