Endonasal endoscopic resection of suprasellar craniopharyngioma: A retrospective single-center case series

IntroductionEndoscopic endonasal approach (EEA) has recently been proposed as an option for resection of primary and recurrent suprasellar craniopharyngioma. However, surgical outcome has not yet been fully evaluated, especially in regards to recurrent cases.MethodsWe analysed our institution (Sir Charles Gairdner University Hospital, Perth, Australia) case-series retrospectively. There were 16 patients operated through an endonasal endoscopic approach from February 2014 to February 2019 for suprasellar craniopharyngiomas. There were 14 primary, and two recurrent lesions. Extent of resection, complications, visual and endocrinological outcomes are presented.ResultsMean age of the patients was 42.9 ± 19.3 years old, with 56% female. The most common clinical symptoms were headaches (9 patients, 56%) and bi-temporal hemianopsia (9 patients, 56%), followed by unilateral optic neuropathy (5 cases, 31%), memory loss (1 case, 6%), hydrocephalus (1 case, 6%), delayed growth and puberty (1 case, 6%), and secondary amenorrhoea (1 case, 6%). Only two cases (12%) initially presented with normal visual function. Gross total resection (GTR) was achieved in 10/16 patients (62.5%), with subtotal resection (STR) in the remainder. Visual symptoms improved in 13/16 patients (81%) and remained unchanged in 3/16 patients (19%). Most common complications included new endocrinological deficit in nine patients (56%), mostly diabetes insipidus, and cerebrospinal fluid leak requiring a new intervention in three patients (19%). There was one mortality case (complicated meningitis, stroke and vasospasm). Mean follow-up time was 22.05 ± 14 months and three patients (19%) had a recurrence of the disease during this period and were referred for radiation therapy.ConclusionEndonasal endoscopic approach is a safe and effective surgical option for both primary and recurrent suprasellar craniopharyngiomas.     

Modern treatment of 84 newly diagnosed craniopharyngiomas

There is debate regarding the appropriate treatment for craniopharyngiomas, which often present symptomatically given their proximity to critical brain structures, and pose significant surgical challenges. The goal of this study is to identify which patient and tumor characteristics are associated with specific preoperative symptoms, surgical complications, patient outcomes, and tumor recurrence in order to guide craniopharyngioma treatment. We retrospectively identified 84 patients with newly diagnosed craniopharyngiomas treated at our institution from 1986–2010. We used binary logistic regression and survival analysis to determine the effect of several variables (including sex, age, tumor size, location, surgical approach, and extent of resection) on preoperative symptoms and postoperative outcomes, including complication rates and tumor recurrence. Age and tumor location were associated with increased rates of preoperative symptoms, with children being more likely than adults to present with endocrine dysfunction, and intraventricular tumors being more likely than extraventricular tumors to present with headaches and hydrocephalus. A transcranial surgical approach was associated with 1.5 times higher rate of surgical complications than transsphenoidal surgery, while only intraventricular tumor location was associated with a poorer patient outcome. The main factor significantly associated with tumor recurrence was extent of resection. We conclude that intraventricular tumor location is most highly correlated with preoperative symptoms. If feasible, transsphenoidal approaches are preferred, as they result in fewer surgical complications, and gross total resections are optimal because they lead to lower rates of recurrence. When gross total resection is not possible, we favor multimodal treatment approaches.     

Premio Sixto Obrador 2013. Modelo topográfico de 3 ejes para el tratamiento quirúrgico de los craneofaringiomas. Parte II: Evidencias anatómicas y neurorradiológicas que definen el modelo de clasificación de 3 ejes y su utilidad para predecir el riesgo quirúrgico individual

Introduction and objectivesThis study evaluates the pathological and magnetic resonance imaging evidence to define the precise topographical relationships of craniopharyngiomas and to classify these lesions according to the risks of hypothalamic injury associated with their removal.Material and methodsAn extensive, systematic analysis of the topographical classification models used in the surgical series of craniopharyngiomas reported in the literature (n = 145 series, 4,588 craniopharyngiomas) was performed. Topographical relationships of well-described operated craniopharyngiomas (n = 224 cases) and of non-operated cases reported in autopsies (n = 201 cases) were also analysed. Finally, preoperative and postoperative magnetic resonance imaging studies displayed in craniopharyngiomas reports (n = 130) were compared to develop a triple-axis model for the topographical classification of these lesions with qualitative information regarding the associated risk of hypothalamic injury.ResultsThe 2 major variables with prognostic value to define the topography of a craniopharyngioma are its position relative to the sellar diaphragm and its degree of invasion of the third ventricle floor. A multivariate diagnostic model including 5 variables –patient age, presence of hydrocephalus and/or psychiatric symptoms, the relative position of the hypothalamus and the mammillary body angle– makes it possible to differentiate suprasellar craniopharyngiomas displacing the third ventricle upwards (pseudointraventricular craniopharyngiomas) from either strictly intraventricular craniopharyngiomas or lesions developing primarily within the third ventricle floor (infundibulo-tuberal or not strictly intraventricular craniopharyngiomas).ConclusionsA triple-axis topographical model for craniopharyngiomas that includes the degree of hypothalamus invasion is useful in planning the surgical approach and degree of resection. Infundibulo-tuberal craniopharyngiomas represent 42% of all cases. These lesions typically show tight, circumferential adhesion to the third ventricle floor, with their removal being associated with a 50% risk of hypothalamic injury. The endoscopically-assisted extended transsphenoidal approach provides a proper view to assess the degree and extension of craniopharyngioma adherence to the hypothalamus.     

颅咽管瘤按发生位置分型和命名——附手术入路与切除方法

目的介绍按颅咽管瘤发生位置来分型方法,并探讨其临床价值。方法回顾性分析215例颅咽管瘤患者的临床资料,根据术前影像学资料、术中发现将颅咽管瘤划分为4种类型,即：Ⅰ型,鞍内颅咽管瘤;Ⅱ型,鞍上颅咽管瘤;Ⅲ型,室下颅咽管瘤;Ⅳ型,室前颅咽管瘤。结果59例Ⅰ型颅咽管瘤全部采用翼点入路切除肿瘤,全切除率为83.1%（49/59）;75例Ⅱ型颅咽管瘤中,74例采用翼点入路,1例采用翼点联合胼胝体入路,全切率为82.7%（62/75）;49例Ⅲ型颅咽管瘤,全部采用翼点入路,全切除率为93.9%（46/49）;32例Ⅳ型颅咽管瘤,全部采用胼胝体入路,全切除率为93.8%（30/32）。结论按发生位置来分型颅咽管瘤是可行的,并使其各自成为相对独立的疾病,更有利于在制定诊疗方案和疗效评价上达成共识。     

前纵裂入路显微手术切除鞍上脑室内外型颅咽管瘤

目的 探讨前纵裂入路在鞍上脑室内外型颅咽管瘤显微手术切除中的应用,并比较与其他手术入路的优缺点.方法 回顾性分析22例采用前纵裂入路显微手术切除鞍上脑室内外型大型颅咽管瘤患者,分析手术技巧及术后效果.结果 肿瘤全切20例,次全切除2例,无手术死亡病例.术后发生电解质紊乱15例,尿量增多17例,2-7周后好转,术后1周内视力好转15例.随访6-60个月,肿瘤复发2例,均为12个月内复发.结论 前纵裂入路可直视下处理肿瘤在垂体漏斗部位的粘连,是鞍上脑室内外型颅咽管瘤较好的手术入路.     

Aggressive resection of craniopharyngioma achieved by drilling the tuberculum sellae/planum sphenoidale using a frontal basal interhemispheric approach: A review of 55 cases

ObjectiveThe frontal basal interhemispheric approach (FBIA) is preferable for resection of craniopharyngioma (CP), achieving desirable total resection rates in early reports of lesions located in the suprasellar region to the third ventricle. For tumours that have created a larger obstruction of the tuberculum sellae and planum sphenoidale, aggressive resection in the intrasellar region and medial wall of the cavernous sinus is not feasible compared to improving tumour visualization by drilling the tuberculum sellae and planum sphenoidale. In a report of drilling the sellar tuberculum and sphenoid planum, drilling allowed the direct visualization of tumours invading the intrasellar region and medial wall of the cavernous sinus. Reconstructing the opening of the sellar-sphenoid cavity is achieved by microsuturing a piece of the pericranium/dura around the dural edge of the defective dura of the open sphenoid sinus and sellar cavity to prevent cerebrospinal fluid (CSF) leakage.Patients and methodsThe FBIA with drilling of the tuberculum sellae and planum sphenoidale was performed to remove the tumours that invaded the intrasellar region and cavernous sinus in 55 patients from January 2014 to October 2019 at our institution. The pre- and postoperative pituitary hormone levels and vision were evaluated as effective standards after surgery and compared using paired t-tests. The different rates of CSF leakage between the packing and microsuture groups were compared by χ² test, p < 0.05.ResultsIn all patients with a mean 37-month follow-up (range, 3–2 months), 43 (78.2%) patients returned to their normal life or school independently, 7 (12.7%) patients were able to perform normal activities with minor complaints or effort, and 4 (7.3%) patients could care for themselves or only required occasional assistance. One (1.8%) death occurred, attributed to CSF leak-related meningitis at 5 months after surgery. Postoperative CSF leakage occurred in eight (19.0%) of 42 patients with packed bone wax or pieces of muscle to the sphenoid sinus. Of 13 patients with a piece of the periosteum/dura microsutured around the defective dura of the sellar region and open sphenoid sinus, one (7.7%) of 13 patients experienced CSF leakage in the perioperative period. With statistical analysis, there was a potential risk for postoperative CSF leakage in the bone wax and muscle piece in the open sphenoid sinus, whereas microsuture manoeuvres were effective for avoiding the risk of postoperative CSF leakage (χ² = 8.865, p < 0.005). The microsutures closed the open sphenoid sinus such that it was water-tight. Postoperative visual acuity and the visual field were not affected by the increased intrasellar exposure or the open sphenoid sinus achieved by drilling the tuberculum sellae and planum sphenoidale.ConclusionTuberculum sellae/planum sphenoidale drilling via FBIA is feasible to enhance the direct visualization of CP resection, which expands the intrasellar region with a direct resection of recurrent tumours in the sellar cavity and adhering to the medial wall of the cavernous sinus. The potential risk of a CSF leakage seemed to be mitigated when using water-tight microsutures on a piece of the pericranium/dura around the edge of the defective dura in the sellar region and the open sphenoid sinus cavity.     

Primary ectopic frontotemporal craniopharyngioma

IntroductionPrimary ectopic craniopharyngiomas have only rarely been reported. Craniopharyngiomas involve usually the sellar and suprasellar region, but can be originated from cell remnants of the obliterated craniopharyngeal duct or metaplastic change of andenohypophyseal cells. We present the first case of a primary ectopic frontotemporal craniopharyngioma.Presentation of caseA 35-year old woman presented with a one-year history of headache and diplopia. MRI showed a large frontotemporal cystic lesion. Tumor resection was performed with a keyhole endoscopic frontal lateral approach. The pathological features showed an adamantinomatous craniopharyngioma with a cholesterol granuloma reaction.DiscussionThere have been reported different localizations for primary ectopic craniopharyngioma. Our case presented a lobulated frontotemporal cystic mass formed by a dense eosinophilic proteinaceous material dystrophic calcifications and cholesterol crystals, with epithelial remnants. No tumor regrowth was observed in the magnetic resonance image 27 months postoperatively.ConclusionPrimary ectopic craniopharyngioma is a rare entity with a pathogenesis that remains uncertain. This is an unusual anatomic location associated with unique clinical findings.     

额下-终板入路切除实质性颅咽管瘤10例报告

目的探讨经额下-终板入路切除实质性颅咽管瘤的显微手术技巧及特点。方法 2007年3月～2010年3月,对10例实质性颅咽管瘤(伴钙化)由额下-终板入路进行手术切除。瘤体全部位于鞍上,其中向三脑室突入7例,向三脑室及鞍后脚间池突入2例,向鞍内生长1例。最长径2.0～5.4 cm,平均3.3 cm。术中充分开放颅底各池,轻轻牵开额下脑组织暴露终板,切开后显露瘤体。结果全切除7例,次全切除3例。术后6例一过性尿崩,7例甲状腺激素低下,半年内均完全缓解。10例术后随访2个月～2年,平均14个月,其中6例>12个月,未见肿瘤复发或增大。结论经额下-终板入路切除鞍上实质性颅咽管瘤具有操作空间大、安全性高、视角佳等优点。术中充分打开脑底各池、解除牵拉额叶所致的各种张力及保护重要组织结构是手术成功及减少术后并发症的关键。