Multiple inflammatory pseudotumor formation after craniopharyngioma resection via an extended nasal endoscopic approach:A case report

BACKGROUND This report describes a case of intracranial multiple inflammatory pseudotumors(IP)after endoscopic resection of a craniopharyngioma,which is relatively rarely reported in the literature,and neurosurgeons should be aware of its existence.CASE SUMMARY Herein,we report the case of a 56-year-old man who developed decreased visual acuity and blurred vision without obvious cause or inducement on April 27,2020.To seek further treatment,he went to the Department of Neurosurgery,Clinical Medical College,Yangzhou University.After falling ill,there was no nausea,vomiting,limb convulsions,obvious disturbance of consciousness,speech disorders,cough,or persistent fever.The neurological examination findings were normal,and pituitary magnetic resonance imaging(MRI)revealed multiple nodules with abnormal signals in the sellar region.The diagnosis was craniopharyngioma.We performed total resection of the tumor via transnasal endoscopy,and the postoperative pathology suggested that the type of tumor was craniopharyngioma.Six months after the operation,the patient experienced sudden hearing loss in the right ear,tinnitus in both ears,and numbness on the right side of the face and head.Meanwhile,cranial MRI showed multiple IP.After steroid hormone and anti-inflammatory therapy,the above symptoms did not significantly improve.Finally,the patient's symptoms were well improved by surgery,and the postoperative pathological diagnosis was multiple IP.CONCLUSION Intracranial inflammatory pseudotumor is a benign disease with slow progression,but the clinical symptoms and imaging findings are not typical,there are no pathological findings,and the diagnosis is relatively difficult.Most of the cases are treated by surgical resection,and the prognosis is good after surgery.

Establishment of primary cultures of craniopharyngioma cells

Craniopharynigoma samples were collected from 36 patients. Out of the 36 samples, 29 achieved successful sub-culturing, with a success rate of 80.6%. Immunohistochemistry staining showed that cytokeratin-7 was positively expressed in the cytomembrane and cytoplasm of craniopharyngioma cells at 6-8 passages, confirming that all cultured cells were squamous epithelial cells. The doubling time of craniopharyngioma cells was 3 days, as confirmed by the 3-（4,5-dimethylthiazol-2-yl）-2,5-diphenyltetrazolium bromide assay. In this study, craniopharyngioma cells cultured in vitro were established; however, establishment of immortalized craniopharyngioma cell lines requires further research.

Cerebral venous sinus thrombosis following transsphenoidal surgery for craniopharyngioma:A case report

BACKGROUND Cerebral venous sinus thrombosis(CVST)is a rare condition in patients with craniopharyngioma following transsphenoidal surgery.CASE SUMMARY A 56-year-old man who underwent transsphenoidal surgery for craniopharyngioma 26 d ago presented gradual headache and cerebrospinal fluid leakage while vomiting 5 d post-discharge and required readmission to our department of neurosurgery.After admission,head imaging examination showed a hyperdense shadow in the superior sagittal sinus and right transverse sinus,edema at the bilateral parietal lobe,and hemorrhage at the left parietal lobe and right occipital lobe;the venous phase of cerebral angiography revealed CVST.The patient was treated immediately by intravenous thrombolysis,endovascular thrombolysis,and mechanical thrombectomy after the definite diagnosis.However,the neurological status of the patient continued to deteriorate and he died on the fourth day after readmission.CONCLUSION For craniopharyngioma undergoing transsphenoidal surgery,it is vital to take an effective strategy to manage the postoperative complications,such as diabetes insipidus,severe electrolyte imbalance,and cerebrospinal fluid leakage.Additionally,the early differential diagnosis of CVST is essential when it develops clinical symptoms,especially in patients following transsphenoidal surgery with a high risk of CVST.Subsequently,the timely and effective treatment of the CVST is critical for preventing neurological deterioration.

PRESERVATION OF THE HYPOTHALAMIC STRUCTURES IN THE TOTAL RESECTION OF CRANIOPHARYNGIOMA

Objective. To explore an effective method of surgical management of craniopharyngioma. Subjects and methods. Fifty patients with craniopharyngioma had total andsubtotal tumor ectomy. There were 29 males and 21 females, ranging in age from 15 to 56 years (mean 34.1 years). MR imaging showed that the tumors were locatedin the superior sellar region in 24 cases, in superior sella region and extended into the third ventricular floor in 19 cases, into parasella in 3 cases and down to intrasella in 4 cases. Complete cystic tumors were found in 5 cases, whilethe partial cystic tumor in 24 cases and complete solid tumors in 21 cases. Pterional approach was used in 48 patients and subfrontal approach in 2 patients. Great attention was paid to the preservation of the perforating arteries from thecarotid, posterior and anterior communicating and anterior choroidal arteries to the hypothalamic structures. The clinical outcome was evaluated according to the GOS scale.Results. Of the 50 patients surgically treated, 47 patients obtained total ectomy of the tumor and 3 patients with the secondary surgery had subtotal ectomy of the tumor. The pituitary stalk was preserved in 29(58%) patients, severed in 14 patients and unidentified in 7 patients. Forty-six patients regained a normal life; one patient needed assist in life. Of the 3 deaths, one patient died of diabetes insipidus, one of inhalation asphyxia, and another one of water and sodium disorders. Conclusion. Avoidance of the injury to the neural structures in the thirdventricular floor and preservation of the perforating arteries to hypothalamus are the key to achieve good surgical results in treating craniopharyngioma.

Transsphenoidal microsurgical treatment of infradiaphragmatic craniopharyngioma

Objective Typically,the transcranial approach has been used for the treatment of craniopharyngiomas with suprasellar extension,whereas the transsphenoidal approach has been used mostly for infradiaphragmatic craniopharyngioma.Total resection of craniopharyngioma can reduce the recurrence rate,especially in young children,but it may lead to severe complications.Therefore,any benefit of the degree of resection must be weighed against the risk of complications by the surgeons.The purpose of this study was to explore the therapeutic outcome after transsphenoidal microsurgical treatment of infradiaphragmatic craniopharyngioma and share our experiences.Methods Between January 2003 and June 2013,30 patients with infradiaphragmatic craniopharyngioma underwent transsphenoidal microsurgical resection in our hospital.The neurological,visual,and endocrine functions,and extent of resection were analyzed retrospectively.Recurrence or growth of residual tumor tissue during follow-up was assessed using magnetic resonance imaging(MRI).Results Total resection was achieved in 25 patients(83.3%),subtotal resection was achieved in 4 patients(13.3%),and partial resection was achieved in 1 patient(3.4%).There were no perioperative deaths.Cerebrospinal fluid(CSF) leakage occurred in 6 patients,and among them,2 required surgical repair of the sella.New-onset postoperative diabetes insipidus(DI) developed in 8 patients.Vision and visual fields were improved at different levels in 13 out of 16 patients who had sight impediments before treatment.Tumor recurrence and regrowth was observed in 2 patients;1 patient underwent transsphenoidal reoperation,the condition of the other patient who had undergone several craniotomies grew worse over the 6-month follow-up period.Conclusion Transsphenoidal surgery is an ideal choice in treating infradiaphragmatic craniopharyngioma.The transsphenoidal approach,which preserves pituitary function and avoids damage to the hypothalamic structures and optic nerve,is associated with fewer complications than the transcranial approach and a low mortality rate.

不经一番寒彻骨怎得梅花扑鼻香:南方医科大学南方医院颅咽管瘤第二本全英文专著Atlas of Craniopharyngioma出版有感

1932年,Harvey Cushing教授最早提出并统一了“颅咽管瘤”这一名词,鉴于颅咽管瘤形态复杂多变、手术难度高且症状多样化,Cushing教授将颅咽管瘤的手术治疗比喻为神经外科技术皇冠上的明珠。之后有学者认为颅咽管瘤存在不可切除(irresectable)类型,并陆续提出各种分型和名词,例如:视交叉前型、视交叉后型;鞍内型、鞍上型、鞍内鞍上型;垂体柄前型、穿垂体柄型、垂体柄后型;严格第三脑室内型、非严格第三脑室内型等。

Surgical Treatment of Craniopharyngiomas in Adults:Comparison between Primary Surgery and Surgery for Recurrence

Objective Few studies have investigated the differences in outcomes between primary and repeat surgery for a craniopharyngioma in adults.As a result,a treatment concept for adult patients with a craniopharyngioma has not yet been established.The present study aimed to retrospectively analyze adult patients with craniopharyngioma to compare surgical outcomes between primary surgery and surgery for recurrence.Methods The demographic and clinical data of 68 adult patients with craniopharyngioma who had primary surgery(n=50)or surgery for recurrence(n=18)were retrospectively analyzed.In addition,the patients were followed up for an average of 38.6 months(range:1–133 months).Results The cohorts of patients undergoing primary surgery or repeat surgery did not differ preoperatively in terms of demographic data,or radiological tumor features.However,patients with recurrent craniopharyngioma had significantly more pituitary hormone deficits and hypothalamo-pituitary disorders before surgery compared with patients with newly diagnosed craniopharyngioma.The success rate of complete resection in primary surgery was 53.2%.Even after repeat surgery,a satisfactory rate of complete resection of 35.7%was achieved.Operative morbidity was increased neither in patients with repeat surgery compared with those with primary surgery(postoperative bleeding P=0.560;meningitis P=1.000;CSF leak P=0.666;visual disturbance P=0.717)nor in patients with complete resection compared with those with partial resection.We found no difference in recurrence-free survival between initial surgery and repeat surgery(P=0.733).The recurrence rate was significantly lower after complete resection(6.9%)than after partial resection(47.8%;P<0.001).Conclusion Attempting complete resection is justified for not only those with newly diagnosed craniopharyngioma but also for those with recurrent craniopharyngioma.However,the surgeon must settle for less than total resection if postoperative morbidity is anticipated.

TREATMENT OF SODIUM DISORDERS AFTER SURGERY OF CRANIOPHARYNGIOMA

Objective.To study the treatment of the patients with blood sodium disorder after craniopharyngioma surgery. Method.The blood sodium in 44 patients with craniopharyngiomas was daily examined from operative to post-operative days. Hypernatremia is defined as [Na]>145 mmol/l, and hyponatremia as [Na]<135 mmol/L. Results.Of the 44 patients, 36 developed sodium disorder. Among them, simple hyponatremia was 16 patients, simple hypernatremia was 9 patients, and alternative sodium disorder was 11 patients. Conclusion.The blood sodium disorder in patients with craniopharyngiomas after surgery appears to present complicated changes. Three types of blood sodium disorder could be determined as simple hyponatremia, simple hypernatremia, and alternative sodium disorder. Treatment of the sodium disorder in patients with craniopharyngiomas after surgery could be carried in accordance with the above three types.

Clinical, Pathological and Surgical Risk Factors Associated with Craniopharyngioma Recurrence: A Literature Review

Objective: This review article attempts to examine and provide an overview of the risk factors associated with craniopharyngioma recurrence. Methods: A literature review of articles relating to the recurrences of craniopharyngioma and the clinical, molecular prognostic indicators of recurrence and treatment outcomes was performed retrospectively. Results: A total of 107 studies which described specific risk factors related to craniopharyngioma recurrence were identified which included but not limited to 54 retrospective case series, 7 systematic reviews, 21 laboratory reports, 13 case reports and 12 literature reviews. Conclusion: Based on the evidence identified in this review, the risk factors for recurrence in craniopharyngioma management are interrelated in a complex way, and surgery with or without adjuvant radiotherapy is reported to be of long-term benefit, but a disparity in findings suggests no definitive consensus on the risk factors of craniopharyngioma recurrence. More high-quality research is needed.

Endoscopic Assisted Microscopic Surgical Removal of Craniopharyngioma

Background: Craniopharyngioma is a benign tumor which represents 2% - 3% of all intracranial tumors, there are two types: childhood type which affects children between 5 and 10 years and adulthood type which affects patients 50 - 60 years old. The presenting symptoms develop over years and include visual, endocrine, hypothalamic, neurological and neurophysiological manifestations. Surgery is the treatment of choice. Postoperative radiotherapy, gamma-knife and intra tumoral injection of chemotherapeutic drugs have been used as an adjuvant therapy in some cases. Objectives: In this study, we evaluated the role of endoscopy in assisting microscopic surgical removal of craniopharyngioma. Methods: Eleven patients were operated upon in Cairo University Hospitals, Egypt. All operations were done using microscope through the subfrontal approach. At the end of surgery, the endoscope was used to detect any residual tumor in the subchiasmatic and retrochiasmatic areas and to visualize the posterior part of the tumor which couldn’t be seen by the microscope to check if it was adherent to the hypothalamus and to evaluate whether to be removed or not. Results: The study included eleven cases, four of which were childhood type and seven adult type craniopharyngiomas. Total removal was achieved in six cases (five cases of adulthood type). Oumaya reservoir was inserted in five cases;ventriculoperitoneal shunt was needed in five cases. All cases suffered from temporary diabetes insipidus postoperatively, while only two cases developed permanent diabetes insipidus. Three cases presented preoperatively with pituitary hypofunction and two cases developed postoperative pituitary hypofunction, which necessitated hormone replacement therapy. Conclusion: Craniopharyngioma is one of the most difficult and challenging tumors for neurosurgeons due to its relation to optic nerve, hypothalamus and vascular system formed by Willis circle and its perforating branches. Endoscopy has a role in decision making after microscopic removal of craniopharyngioma.

Microsurgical Treatment of Craniopharyngiomas: A Retrospective Analysis

Objective: This retrospective study aims to survey the clinical outcomes of 341 consecutive patients surgically treated for and diagnosed with craniopharyngioma (CP) treated in a 10-year period in a single institution. Methods: The clinical reports of three hundred forty-one patients CP patients treated surgically between January 2006 and December 2016 were reviewed and analyzed retrospectively. Results: Our cohort consisted of 341 patients (202 male, 139 female) with a mean age of 34.9 years (range 1 - 74 years);Tumor Features: 129 patients (37.8%) had cystic tumors, 88 (23.8%) had solid tumors whereas 126 (36.4%) had heterogeneous lesions with a solid and cystic portion;calcifications were present in 139 (40.8%);Tumor Topography: Suprasellar 198 (58.1%), Intrasellar 40 (11.7%), Intra-third ventricular 103 (30.2%);Surgical approaches used among the patients included: Pterional 262 (76.8%), Transsphenoidal (TS) 42 (12.3%) Transcallosal 20 (5.9%), Transcortical 16 (4.7%) suboccipital 1 and combined approach 1;Gross total removal (GTR) was achieved in 247 patients (72.4%), Subtotal removal (STR) in 94 patients (27.6%). Good postoperative outcome at discharge was achieved in 324 (95%) patients while 17 (5%) patients had poor outcome including 5 (1.5%) perioperative deaths. Mean hospital length of stay was 21.87 (8 - 129). There were 42 (12.9%) recurrences with a mean time to recurrence of 28.36 (3 - 84) months, among which 37 (88.1%) underwent surgery for recurrence treatment. Follow-up time ranged from 3 months to 10 years. There was a statistical significance between open transcranial surgery and suprasellar tumors (p < 0.0001), TS and intrasellar tumors (p < 0.0001);postoperative diabetes inspidus and gross total resection (p < 0.0001);GTR and cystic tumors (p = 0.034) calcification and GTR (p = 0.0008). Conclusion: Good surgical outcome and long-term tumor control can be achieved through individual-based selective resection, whether total or subtotal resection. Whereas surgical prehistory of CP was found to be a significant risk factor to recurrence (p < 0.0001), the clinical risk factors of CP of recurrence are still arguable, including the presence residual tumor due to subtotal resection. More future studies are necessary.

Multi-omics analysis of adamantinomatous craniopharyngiomas reveals distinct molecular subgroups with prognostic and treatment response significance

Background:Adamantinomatous craniopharyngioma(ACP)is the commonest pediatric sellar tumor.No effective drug is available and interpatient heterogeneity is prominent.This study aimed to identify distinct molecular subgroups of ACP based on the multi-omics profiles,imaging findings,and histological features,in order to predict the response to anti-inflammatory treatment and immunotherapies.Methods:Totally 142 Chinese cases diagnosed with craniopharyngiomas were profiled,including 119 ACPs and 23 papillary craniopharyngiomas.Whole-exome sequencing(151 tumors,including recurrent ones),RNA sequencing(84 tumors),and DNA methylome profiling(95 tumors)were performed.Consensus clustering and non-negative matrix factorization were used for subgrouping,and Cox regression were utilized for prognostic evaluation,respectively.Results:Three distinct molecular subgroups were identified:WNT,ImA,and ImB.The WNT subgroup showed higher Wnt/β-catenin pathway activity,with a greater number of epithelial cells and more predominantly solid tumors.The ImA and ImB subgroups had activated inflammatory and interferon response pathways,with enhanced immune cell infiltration and more predominantly cystic tumors.Mitogen-activated protein kinases(MEK/MAPK)signaling was activated only in ImA samples,while IL-6 and epithelial-mesenchymal transition biomarkers were highly expressed in the ImB group,mostly consisting of children.The degree of astrogliosis was significantly elevated in the ImA group,with severe finger-like protrusions at the invasive front of the tumor.The molecular subgrouping was an independent prognostic factor,with the WNT group having longer event-free survival than ImB(Cox,P=0.04).ImA/ImB cases were more likely to respond to immune checkpoint blockade(ICB)therapy than the WNT group(P<0.01).In the preliminary screening of subtyping markers,CD38 was significantly downregulated in WNT compared with ImA and ImB(P=0.01).Conclusions:ACP comprises three molecular subtypes with distinct imaging and histological features.The prognosis of the WNT type is better than that of the ImB group,which is more likely to benefit from the ICB treatment.

Ectopic recurrence craniopharyngioma:series report and literature review

Background Craniopharyngioma is a common intracranial tumor located in the sellar-suprasellar region.Due to the involvement of adjacent structures,it can lead to increased intracranial pressure,visual impairment,and endocrine deficiencies.Surgical resection is the primary treatment,but it is a tough challenge to achieve total resection,which will led to the frequency of recurrences and progressions.Among them,distant spread is extremely rare,but important complication,identifying and providing proper therapy,is crucial.Methods We report two cases of ectopic recurrence craniopharyngioma and make a literature review for the published similar case reports.Results Our literature review revealed 63 cases(including our patient).The onset age in children group and adult group ranges from 2-14 years old(6.70±3.33)to 17-73 years old(40.63±15.58),while the interval year between tumor initiation and ectopic recurrence ranges from 0.17-20(7.28±6.76)years to 0.3-34(6.85±7.29).Achieving gross total resection seems not to prevent the ectopic recurrence.The major pathology of ectopic recurrence craniopharyngioma is adamantinomatous type.The most common site of ectopic recurrence is frontal lobe.According to the pathogenesis,35 cases were seeding along the surgical approach,and 28 cases were seeding via the CSF pathway.Conclusion Ectopic recurrence craniopharyngioma is rare,but it can lead to serious symptoms.Delicate surgical procedure can help to reduce the risk of ectopic recurrence,and standardized follow-up can provide valuable information for treatment.

误诊为颅烟管瘤的垂体母细胞瘤一例分析并文献复习

回顾性分析1例垂体母细胞瘤误诊为颅烟管瘤病例的临床、影像、病理及治疗经过,以提高鞍区病变的鉴别诊断能力。本例患者年龄为2岁,以右侧下肢无力2周,右侧上肢无力3天为主诉入院,无明显诱因出现走路不稳,无哭闹、跌倒,无发热、头痛、呕吐,无抽搐、惊厥。右侧膝、跟腱反射稍减弱,右侧Babinski's征可疑阳性。功能评价提示中枢性尿崩症和垂体前叶功能低下。磁共振表现为鞍内及鞍上囊实性占位,诊断为颅烟管瘤。经手术切除后病理诊断为垂体母细胞瘤。对于年龄较小,鞍内及鞍上占位,疑诊为颅烟管瘤或垂体瘤的患者,应尽早活检或手术以明确诊断。

神经内镜经鼻经蝶入路手术切除颅咽管瘤的临床效果及安全性

目的探讨神经内镜经鼻经蝶入路手术切除颅咽管瘤的临床效果及安全性。方法选择2020年3月至2023年4月郑州大学第一附属医院收治的76例颅咽管瘤患者,根据随机数字表法分为治疗组与参考组,各38例。治疗组行神经内镜经鼻经蝶入路手术治疗,参考组行经颅入路手术治疗。对比2组手术指标、临床疗效、并发症发生情况。结果治疗组术中出血量少于参考组,手术时间、切除肿瘤时间、住院时间较参考组短,有统计学差异(P<0.05)。治疗组治疗总有效率为97.37%(37/38),高于参考组的78.95%(30/38),有统计学差异(P<0.05);治疗组并发症总发生率为2.63%(1/38),低于参考组的23.68%(9/38),有统计学差异(P<0.05)。结论神经内镜经鼻经蝶入路手术治疗颅咽管瘤可提高疗效,缩短手术时间,减少出血量与并发症发生,利于加快患者康复速度,安全有效。

颅咽管瘤相关下丘脑性肥胖危险因素及饮食运动干预研究进展

颅咽管瘤相关下丘脑性肥胖已成为颅咽管瘤患者术后管理中的重要问题。有研究指出通过调整热量摄入、制订并实施运动计划能显著降低颅咽管瘤相关下丘脑性肥胖患者体质量,可将非药物治疗措施如饮食运动干预应用于颅咽管瘤患者的体质量管理中,但目前国内相关研究较少。因此,本文综述了颅咽管瘤相关下丘脑性肥胖现状、危害、危险因素和饮食运动干预策略,以期改善疾病预后、提高生存质量。建议临床医护人员应重视颅咽管瘤患者相关下丘脑性肥胖,开展颅咽管瘤相关下丘脑性肥胖患者体质量控制的前瞻性研究,并构建科学严谨的饮食运动干预方案,为全面提高颅咽管瘤患者的生存质量提供参考。

单纯鞍下恶性颅咽管瘤一例报告及文献回顾

本文报道单纯位于鞍下恶性颅咽管瘤1例。患者男性,60岁,因2月前无明显诱因出现头痛入院。CT表现为鞍下区等密度软组织肿块,颅底斜坡骨质破坏。MR表现为鞍下区不规则实性肿块,T_(1)WI呈均匀低信号,T_(2)WI呈稍高信号,DWI呈均匀低信号,T_(2)WI和T_(1)WI增强序列均表现为高低相间混杂信号的“椒盐征”。术后见鞍内垂体结构完整,鞍上及鞍内未侵犯。最终病理诊断为恶性颅咽管瘤(鳞形细胞癌)。

儿童和青少年颅咽管瘤术后内分泌管理专家共识

颅咽管瘤是发生于儿童鞍区的少见良性肿瘤,术后患者可长期存活。因肿瘤及手术常损害下丘脑和垂体功能,导致下丘脑综合征、垂体前叶功能减退症、肥胖、电解质紊乱等一系列功能异常,需内分泌科为主的多学科团队进行长期的精细的激素治疗和随访管理。本共识针对此类人群,围绕围手术期管理、肥胖管理、激素替代、身高增长和青春发育等主题,制定规范化的长期随访管理建议,以提高临床医生的应对水平和患者的健康状态。

颅咽管瘤分型

颅咽管瘤是常见的颅内良性肿瘤,可能起源于垂体柄任何部位。肿瘤的不同起源部位与其生长模式相关,也与肿瘤与鞍上及周围神经血管结构之间的关系相关。目前针对颅咽管瘤有多种不同的分型方式,依据肿瘤起源部位、解剖部位、影像学特征等。本文通过回顾总结不同颅咽管瘤的分型方式,尝试探究肿瘤起源、生长模式、影像学特点及其与微观结构之间的关系;通过影像学特点分析肿瘤起源,判断其与周围组织的粘连情况,以指导手术方式及判断预后。

颅咽管瘤相关下丘脑性肥胖患儿饮食运动管理方案的构建

目的构建颅咽管瘤相关下丘脑性肥胖患儿饮食运动管理方案,为临床实践提供参考。方法以健康行为改变整合理论为指导,通过文献循证、半结构式访谈,形成颅咽管瘤相关下丘脑性肥胖患儿饮食运动管理方案初稿;运用德尔菲法,遴选19名专家对方案进行2轮函询,完善并确定最终方案。结果2轮专家函询问卷回收率分别为90.48%、100%,专家权威系数分别为0.89、0.90;第2轮函询中,各指标重要性变异系数0.06~0.19,肯德尔和谐系数0.101(P<0.05),各指标可行性变异系数0.10~0.23,肯德尔和谐系数0.122(P<0.05);最终形成包含4个一级指标、18个二级指标、44个三级指标的颅咽管瘤相关下丘脑性肥胖患儿饮食运动管理方案。结论颅咽管瘤相关下丘脑性肥胖患儿饮食运动管理方案具有较好的专家认可度和可行性,可进一步开展临床验证研究。