Craniopharyngioma In Children Research Articles

Evaluation of postoperative fluctuations in plasma sodium concentration and triphasic response after pediatric craniopharyngioma resection: A French cohort study.

Disturbances in plasma sodium levels are a major complication following recent resections of craniopharyngiomas in children. They must be properly managed to avoid neurological sequelae. We aimed to describe the variations and characteristics of postoperative natremia in children who had undergone a first craniopharyngioma resection with a particular focus on the frequency of triphasic syndrome in these patients. Paediatric patients with craniopharyngiomas who underwent a first surgical resection in the neurosurgery department of the Hôpital Femme Mère Enfant (Lyon, France) between January 2010 and September 2021 were included in the present study and the medical records were analysed retrospectively. A total of 26 patients were included. Of these, 17 (65.4%) had a postoperative course characterised by the occurrence of both initial diabetes insipidus (DI) and hyponatremia a few days later. Eight patients (30.8%) presented then with isolated and persistent DI. Patients with the triphasic syndrome had a significantly higher grade of Puget classification on MRI (1 and 2), compared to the other patients. Dysnatremia is common after craniopharyngioma resections in children. This immediate postoperative complication is particularly difficult to manage and requires rapid diagnosis and prompt initiation of medical treatment to minimize fluctuations in sodium levels and avoid neurological sequelae.

Risk-benefit analysis of surgical treatment strategies for cystic craniopharyngioma in children and adolescents.

Treatment strategies for craniopharyngiomas are still under debate particularly for the young population. We here present tumor control and functional outcome data after surgical treatment focusing on stereotactic and microsurgical procedures for cystic craniopharyngiomas in children and adolescents. From our prospective institutional database, we identified all consecutive patients less than 18 years of age who were surgically treated for newly-diagnosed cystic craniopharyngioma between, 2000 and, 2022. Treatment decisions in favor of stereotactic treatment (STX) or microsurgery were made interdisciplinary. STX included aspiration and/or implantation of an internal shunt catheter for permanent cyst drainage. Microsurgery aimed for safe maximal tumor resections. Study endpoints were time to tumor recurrence (TTR) and functional outcome including ophthalmological/perimetric, endocrinological, and body-mass index (BMI) data. 29 patients (median age 9.9 yrs, range 4-18 years) were analyzed. According to our interdisciplinary tumor board recommendation, 9 patients underwent stereotactic treatment, 10 patients microsurgical resection, and 10 patients the combination of both. Significant volume reduction was particularly achieved in the stereotactic (p=0.0019) and combined subgroups (p<0.001). Improvement of preoperative visual deficits was always achieved independent of the applied treatment modality. Microsurgery and the combinational treatment were associated with higher rates of postoperative endocrinological dysfunction (p<0.0001) including hypothalamic obesity (median BMI increase from 17.9kg/m2 to 24.1kg/m2, p=0.019). Median follow-up for all patients was 93.9 months (range 3.2-321.5 months). Recurrent tumors were seen in 48.3% and particularly concerned patients after initial combination of surgery and STX (p=0.004). In here, TTR was 35.1 ± 46.9 months. Additional radiation therapy was found indicated in 4 patients to achieve long-lasting tumor control. In children and adolescents suffering from predominantly cystic craniopharyngiomas, stereotactic and microsurgical procedures can improve clinical symptoms at low procedural risk. Microsurgery, however, bears a higher risk of postoperative endocrine dysfunction. A risk-adapted surgical treatment concept may have to be applied repeatedly in order to achieve long-term tumor control even without additional irradiation.

An update on multimodal management of craniopharyngioma in children.

Craniopharyngioma (CP) represent 1.2-4.6% of all intracranial tumors in children and carry a significant morbidity due to their lesional intimacy with structures involved in neurological, visual, and endocrinological functions. Variable treatment modalities being available, including surgery, radiation therapy, alternative surgeries, and intracystic therapies or combinations of them, their common goal is to reduce immediate and long-term morbidity while preserving these functions. Multiple attempts have been made to re-evaluate surgical and irradiation strategies in order to optimize their complication and morbidity profile. However, despite significant advances in "function sparing" approaches, such as limited surgery and improved technologies of radiation therapies, achieving interdisciplinary consensus on the optimal treatment algorithm remains a challenge. Furthermore, there remains a significant span of improvement given the number of specialties involved as well as the complex and chronic nature of CP disease. This perspective article aims to summarize recent changes and knowledge gains in the field of pediatric CP, outlining updated treatment recommendations, a concept of integrative interdisciplinary care and the implication of novel potential diagnostic tools. A comprehensive update on the multimodal treatment of pediatric CP is presented, focusing on "function-preserving" therapies and their implications.

Endoscopic endonasal approach for craniopharyngiomas in children

Background. The incidence of craniopharyngiomas is up to 15 % of intracranial tumors in children. The endoscopic endonasal approach (EEA) is widely spread nowadays and especially for the craniopharyngiomas removal in adults. Nevertheless, this approach is still not so common in pediatric patients. The endoscopic technique significantly improves the visualization of the structures located around the tumor.Aim. To determine the possibilities and limitations for the use of EEA in the treatment of pediatric patients with cranio pharyngiomas.Materials and methods. A total five pediatric patients with craniopharyngiomas were operated via EEA Federal Center of Neurosurgery of Ministry of Health of Russia (Novosibirsk): the age of patients ranged from 4 to 17 years, including 3 boys and 2 girls. All patients at the preoperative stage already had endocrine disorders in one volume or another. Postoperative evaluation of the visual functions showed the improvement in one patient, three patients remained unchanged and one patient developed worthening. In addition, the volume of the sphenoid sinus was studied, the degree of pneumatization of which determines the anatomical accessibility of the tumor.Results. Our experience of the EEA for the craniopharyngiomas in pediatric patients has shown a positive result regarding to the tumor’s gross‑total removal and no data of the tumor recurrence at the follow‑up. According to our results it can be judged that endonasal endoscopic approach for the craniopharyngiomas in pediatric patients could be a method of choice based on the anatomical accessibility of the tumor and its relationships with the critical neurovascular structures.Conclusions. Endoscopic endonasal removal of craniopharyngiomas in pediatric patients can be successfully used when the tumor is anatomically accessible. The experience of using EED in surgery for craniopharyngiomas in children has shown positive results, including a high radical removal of the tumor and the absence of relapses during further follow‑up of patients.

Transnasal endoscopic surgery of craniopharyngiomas in children

Transnasal endoscopic surgery of craniopharyngiomas in children

Craniopharyngioma in Children and Adults – Does the Age Matter?

<h3>Purpose/Objective(s)</h3> To investigate the long-term results of treatment of children and adults with craniopharyngioma and to identify factors related to survival. <h3>Materials/Methods</h3> A retrospective analysis of all consecutive patients (pts) treated due to craniopharyngioma in years 1979–2019 was performed. Event-free survival (EFS) was defined as no tumor progression or pts` death after the end of treatment (radiotherapy – RT or the last surgery in non-irradiated pts). Standard statistical tests were used. <h3>Results</h3> The group consisted of 97 pts (41 children, 56 adults) with median age of 26 years (range 2-71) and small predominance of males (56%). The most common symptoms were visual disturbances (54%), headaches (54%), endocrine disorders (20%) and nausea/vomiting (22%). The diagnosis was based on histopathological examination in the majority of pts (84%) with remaining 16 cases diagnosed based on characteristic radiological features. Surgery was the primary treatment in 94% with majority having one (44%) or two (26%) surgeries, while 3, 4 and 5 surgeries were conducted in case of 14%, 6% and 4% of pts, respectively. The most common deficits observed after surgery were visual disturbances (45%) and endocrine disorders (62%). RT was applied in 89% pts with 63% receiving irradiation as part of salvage treatment. Conventional RT (CRT) and stereotactic RT (SRT) was applied in 58 and 28 pts, respectively. Median total dose (TD) in CRT was 54 Gy and 16 Gy in SRT. Ten patients in CRT group had stereotactic boost (median TD 6 Gy). The most common side effects of RT were endocrine disorders (14%), visual disturbances (7%), vasculopathies (6%) and deterioration in general status (8%). Secondary tumors within RT volume were observed in 3 pts (2 high grade gliomas, 1 meningioma). Median follow-up was 13 years from the diagnosis and during that time 29 pts died. Five- & 10-year overall survival (OS) was 90% & 78%, respectively. Univariate analysis showed that adults had worse OS compared to children (p=0.001) with 5- & 10-year OS of 85% & 68% vs 97% & 92% for adult vs children, respectively. Also, pts who did not receive RT had inferior outcome with 5- and 10- year OS of 82% & 34% vs 92% & 84% (p=0.0006). In multivariate analysis, only age was an independent factor affecting OS (p=0.005). Median EFS was 8.4 years. Five- and 10- year EFS for the whole group was 78% and 62%. Univariate analysis showed that adults had worse EFS compared to children (p=0.048). RT was also found in univariate analysis to have positive impact on EFS (p=0.021) which was also confirmed in multivariate Cox regression model (p=0.037). The local effect of RT was evaluated in case of 78 pts with stagnation & regression observed in 35 & 37 pts. The comparison of CRT with SRT showed inferior local control in SRT group (p=0.023). However, in SRT arm, in 5 out of 8 failures TD applied was ≤15 Gy. <h3>Conclusion</h3> Adjuvant RT should not be omitted as it is associated with better local control. Meticulous follow up is required to assure effective salvage treatment in case of progression.

Upfront adjuvant irradiation versus postoperative surveillance following incomplete surgical resection of craniopharyngiomas in children and young adults.

Incomplete surgical removal of craniopharyngiomas frequently results in suboptimal oncological control. Radiation therapy is usually offered in these cases to prevent local recurrence of disease; however, the efficacy of radiation is limited by its potential adverse effect, particularly in younger patients. This study was undertaken to compare long-term outcomes and rates of postoperative obesity and endocrinopathy in patients undergoing either upfront adjuvant radiation after surgery, or postoperative surveillance with progression-contingent intervention. Thirty-seven patients aged <25 years who had undergone primary incomplete surgical resection of craniopharyngiomas were retrospectively identified and categorized according to the prescribed treatment strategy. Recurrence rates, functional status, neuro-ophthalmologic, and endocrine outcomes were studied in both groups of patients. Twenty-three patients received upfront adjuvant radiation, and 14 patients underwent postoperative surveillance. Adjuvant radiation in the former group was delivered using either conventional (n=10), 3D-conformal (n=4), or fractionated stereotactic (n=9) techniques using a linear accelerator. The mean follow-up duration was 64.7 months (range 14-134 months). Disease progression was significantly higher in patients undergoing surveillance as compared to those undergoing upfront adjuvant radiation (71.4 versus 17.4%; p=0.002). Median progression-free survival times were 129 months and 27 months in the upfront adjuvant radiation and surveillance groups, respectively (p=0.007). In patients undergoing surveillance, 50% ultimately required irradiation, and the median radiation-free survival time in this subgroup was 57 months. Two children in the adjuvant radiation group developed asymptomatic radiation-related vasculopathies on follow-up; however, there were no statistically significant differences between the two groups in terms of visual, functional, or pituitary-hypothalamic function at last follow-up. In comparison to upfront adjuvant radiation following incomplete craniopharyngioma resection significantly, a strategy of postoperative surveillance resulted in less durable disease control but allowed radiation therapy to be delayed by a median time of 57 months, without significant detriment to global functional, visual, and neuro-endocrinological outcomes. The merits and demerits of either strategy should be carefully considered in the post-surgical management of these patients.

Extended Neuroendoscopic Endonasal Approach for Resection of Craniopharyngioma in Children.

ObjectiveTo explore the surgical approach and technique of neuroendoscopic endonasal resection of pediatric craniopharyngiomas and to further evaluate its safety and effect in children.MethodsThe clinical data of 8 children with craniopharyngiomas who were surgically treated by neuroendoscopy through an extended endonasal approach in our center from 2018 to 2021 were retrospectively analyzed. The related surgical approach and technique were evaluated to improve the surgical results and further reduce the surgical complications when removing craniopharyngioma in children.ResultsAll 8 patients achieved a gross-total resection of the tumor under neuroendoscopy. Postoperatively, 2 cases of transient hyperthermia and 4 cases of transient hyper- and/or hyponatremia occurred within the first 2 weeks, all of which were quickly controlled. Seven patients had symptoms of diabetes insipidus to varying degrees after the operation, and 4 of them improved within 1–3 months after surgery, but 3 cases still needed oral pituitrin. There were no cases of coma or death, leakage of cerebrospinal fluid, or severe electrolyte imbalance after surgery. During the postoperative follow-up of 3 months to 2 years, no tumor recurrence was found. Among the 7 patients who suffered postoperative neuroendocrine deficiencies, 3 patients were found to be temporary during the follow-up, but 4 patients still required hormone replacement therapy. Particularly, postoperative visual deterioration and olfactory defect that occurred in patients were all improved during follow-up periods. In addition, 4 cases of obesity were noted at the last follow-up.ConclusionsExtended neuroendoscopic endonasal resection of craniopharyngiomas may be used as a safe and effective approach for children. Due to the poor pneumatization of the sphenoid sinus and worse compliance of treatment in children, surgical techniques of exposing the sellar region, removing the tumor, and reconstructing the skull base, as well as postoperative management of patients was proposed. However, due to the limited surgical cases in the study, the surgical safety and effects of the extended neuroendoscopic endonasal approach for children with craniopharyngiomas need to be further studied in the future.

Optical Coherence Tomography Angiography Predicts Visual Outcomes for Craniopharyngioma in Children by Quantifying Choroidal Capillaries.

Purpose: To predict the prognosis of craniopharyngioma in children by optical coherence tomography angiography (OCTA).Methods: We evaluated if the relationship between preoperative OCTA of the choroidal capillary density (CCD) and visual outcome continued over long-term visual recovery in 38 patients undergoing craniopharyngioma resection. Patients were evaluated 3 times: 1 week before surgery (Visit1), followed-up at 6–10 weeks (Visit2), and 9–15 months (Visit3) after surgery.Results: In total 38 patients (70 eyes) with craniopharyngiomas, which included 20 (52.6%) boys and 18 (47.4%)girls, the mean age was 11.8 ± 2.7 years (range: 6–18 years). The age (p = 0.71), gender (p = 1.00), mean refractive error (p = 0.55), and axial length (p = 0.23) of 38 normal volunteers (76 eyes) were matched. After surgery, the cross-compression of patients was relieved. The average visual acuity change in the normal CCD group was 0.07 ± 0.02; the average visual acuity change in the low CCD group was 0.01 ± 0.01, p < 0.001. Preoperative CCD value is related to the preoperative BCVA (p < 0.001), and the visual function after the long-term follow-up (9–15 months) (p < 0.001). The prognosis of CCD has the same trend as the BCVA. Further correlation analysis shows that CCD and BCVA are significantly correlated (r = 0.878; p < 0.001). CCD has a weak but significant correlation both with MD (r = 0.19; p < 0.001) and PSD (r = −0.21; p <0.001). A natural cutoff of CCD is approximately 38%. With the normal CCD group the maximum improvement of BCVA exceeds 0.3 post-operatively, compared to eyes in the low CCD group that improve by <0.03, and worse after surgery.Conclusions: Long-term vision recovery after surgical decompression of craniopharyngiomas in children can be predicted by preoperative by OCTA. Patients with normal CCD before surgery showed a tendency to improve vision; this trend of improvement persisted in subsequent follow-ups. The CCD baseline natural cutoff value for predicting visual prognosis before and after surgery is about 38%.

Letter to the Editor. Surgical management and long-term outcomes of craniopharyngioma in children.

Letter to the Editor. Surgical management and long-term outcomes of craniopharyngioma in children.

Craniopharyngioma in children: Report of three cases and review of the literature

Craniopharyngioma in children: Report of three cases and review of the literature

Craniopharyngioma in children: Report of three cases and review of the literature

Craniopharyngioma is a rare benign epithelial tumor, originating in the pituitary stem or pituitary gland and developing in the sellar and / or suprasellar region. Of embryonic origin, craniopharyngiomas are considered to develop from epithelial remains of Rathke's pouch. It affects children as well as adults. All ages combined, craniopharyngiomas represent between 3 to 4% of intracranial tumors worldwide, or 0.5 to 2 new cases per year and per million inhabitants. In children, they represent 10% of all intracranial tumors with a peak frequency between 7 and 13 years and a predominance of men. Although benign, this tumor remains a serious pathology because of the frequent visual, endocrine, neuro-intellectual sequelae and the risk of recurrence that it entails, involving the visual and vital functional.The management of this pathology is multidisciplinary and involves several modalities such as surgery, radiotherapy and medical treatment of hormonal deficits very often associated. We report in this article three cases of boys aged 9, 10 and 12; treated for symptomatic craniopharyngiomas with a favorable clinical and radiological outcome after partial surgical excision and adjuvant external radiotherapy. The aim of this article is to review the epidemiological, diagnostic, therapeutic and evolutionary aspects of these benign tumors of the sellar and / or suprasellar region, with an emphasis on the interest of radiotherapeutic treatment.

Child's Craniopharyngiomas About 11 Cases and Review of the Literature

Craniopharyngioma is a benign and rare intracranial tumor. In children, the clinic is characterized by the semiological, neurological, ophthalmological, and endocrine tripod. Medical imaging, represented by computed tomography (CT) and especially magnetic resonance imaging (MRI), has revolutionized the approach to the diagnosis of the latter. It has a slow evolution and likely to recur late. Thus, prolonged clinical-radiological follow-up is important to affirm a cure. The aim of this study is to highlight the epidemiological, clinical, paraclinical, therapeutic and evolutionary characteristics of craniopharyngiomas in children. This work consisted of a retrospective study of 11 pediatric cases of this tumor collected in the Neurosurgery Department of the IBN ROCHD University Hospital in Casablanca between January 2011 and January 2017. In this series, the child's craniopharyngiomas accounted for a rate of 45.8% of all these tumors. The average age of our patients was 9.09 years, with a sex ratio of 0.37. The average time to diagnose was 10 months. The picture was dominated by 100% visual disturbances, signs of intracranial hypertension at 63.63% and endocrine disorders at 36.36%. The lesion was intra and suprasellar seat in 91% of cases and suprasellar seat in 9%. Tumor size was less than 4cm in 54.53% of cases. Hydrocephalus is observed in 27.27% of cases. The fronto-pterrional modus operandi was the most used 72% of cases. Total exeresis was achieved in 9.1% of cases. Overall trends were favorable in 72% of cases without noting mortality. Tumor progressive recovery was observed in 9.1% of cases, but there were no recurrences. Given its location, the treatment of craniopharyngioma can lead to significant consequences in children affecting the long-term quality of life, hence the importance of post-operative follow-up and multidisciplinary management.

Management Options for Suprasellar Cystic Craniopharyngioma: Endoscopic Transventricular Approach and Microsurgical Approach

Objective The treatment of cystic craniopharyngioma in children is varied. The treatment ranges from radical excision to direct radiotherapy. As the morbidity of excision is high, more conservative approaches are used. Transventricular endoscopy is a minimally invasive treatment for cystic craniopharyngiomas. The objective of this study is to compare the outcome of microscopic versus endoscopic transventricular approach for cystic craniopharyngioma. Methods This is a retrospective study of series of children managed with microscopic excision and endoscopic transventricular approach for suprasellar cystic craniopharyngiomas. Operative details, visual outcome, endocrinological outcome, tumor-related cyst recurrence rate, and complication were compared between microscopic and endoscopic groups. Results A total of 28 children underwent microscopic excision and 14 children underwent endoscopic procedure. The anesthesia time was significantly lower with endoscopic as compared to microscopic group ( p = 0.0001) as well as blood loss during surgery ( p = 0.0001). Hospital stay after surgery was shorter in endoscopic group. Incidence of diabetes insipidus was more in microscopic group (25%) compared to endoscopic group (7.14%). Visual outcome was almost same with approaches. Requirement of hormone replacement was more in microscopic group than in endoscopic group ( p = 0.006). Incidence of cyst recurrence was more in microscopic (39.3%) compared to endoscopic group (7.7%). Conclusion Endoscopic transventricular approach is a safe alternative for initial treatment of suprasellar cystic craniopharyngioma in children.

Interferon-alfa in the management of cystic craniopharyngioma in children under 5 years of age

Introduction: the best therapeutic option for the management of craniopharyngioma in younger children remains controversial, ranging from complete surgical resection, partial surgical resection associated with radiotherapy and application of chemotherapeutic agents such as bleomycin and interferon-alfa. Objective: to verify the response to treatment with interferon-alfa via Ommaya reservoir in a group of children under 5 years of age with diagnosis of cystic craniopharyngioma. Methods: description of a case series through consecutive review of medical records of children under 5 years of age diagnosed with cystic adamantinomatous craniopharyngioma who had an Ommaya reservoir catheter surgically implanted for intratumoral application of interferon-?. Results: seven children aged 18 to 60 months (median 46 months) with the abovementioned diagnosis and treated with interferon-alfa between 2010 and 2019, according to a pre-established protocol, were identified. A reduction in tumor volume, ranging from 88 to 100%, one year after the end of treatment was observed in the study sample. There were no complications that justified the interruption or modification of the established therapy. Conclusion: in all the cases evaluated of children less than 5 years of age with predominantly cystic adamantinomatous craniopharyngioma we observed a reduction of tumor volume on magnetic resonance imaging one year after the end of treatment with interferon-alfa.

RARE-63. CYST WALL OF ADAMANTINOMATOUS CRANIOPHARYNGIOMA CONTAINS TUMOR CELLS THAT COULD LEAD TO RECURRENCE AFTER SURGERY

BACKGROUNDAdamantinomatous craniopharyngioma (ACP) is the primary subtype of craniopharyngioma in children, frequently with mutations in exon 3 of the CTNNB1 gene. Most ACP consists of both a solid tumor and one or more cysts. Despite surgical resection of the solid tumor followed by radiation, recurrence involving the cystic component is common, suggesting that the cyst wall contains tumor cells. We present here conclusive molecular pathology evidence of the presence of tumor cells in the cyst wall similar to those in the solid tumor.METHODSWe used standard H&E staining and immunohistochemistry (IHC) to compare the histopathology characteristics between the matched cyst wall and solid tumor of 11 cases of ACP as well as their CTNNB1 expression and exon 3 mutation.RESULTSSamples of the cyst wall and solid tumor were collected separately during the operation of 11 cases of ACP through careful dissection. The cyst wall showed the nested cell clusters and peripheral palisading epithelium which are identical to those in the solid tumor. The cyst wall and solid tumor both showed Ki67 and nuclear β-catenin expression by IHC. There is no difference in the transcription level of CTNNB1 between the cyst wall and the solid tumor, both being significantly higher than that in normal brain tissue. Exon 3 mutations of the CTNNB1 gene of the cyst wall and the solid tumor are identical in each case.CONCLUSIONFollow-up of clinical cases suggests that tumor cells in residual cyst wall may be the cause of recurrence after surgery.

儿童颅咽管瘤的治疗方法及其疗效现状分析

儿童颅咽管瘤的治疗方法及其疗效现状分析

Craniopharyngioma in children: trends from a third consecutive single-center cohort study.

The management of children with craniopharyngioma has evolved over time, with a trend toward less invasive neurosurgical approaches as surgeons have sought to balance oncological control and treatment-related morbidity. To this end, the aim of this study was to evaluate the safety and effectiveness of the current management of children with craniopharyngioma compared to the previous management methods used at the authors' treatment center. A prospectively maintained database was searched over a 14-year period between January 1, 2005, and December 31, 2018, to identify all children 17 years of age or younger with a new diagnosis of craniopharyngioma. A retrospective case note review was performed for each child to extract data on the presentation, investigation, treatment, and outcome of their illness. Morbidity was assessed in the same fashion as in previous cohorts, according to the following categories: visual loss, pituitary dysfunction, hypothalamic dysfunction, neurological deficits, and cognitive impairment. In total, 59 children were identified with craniopharyngioma during the study period. A total of 92 operations were performed, including cyst drainage (35/92; 38.0%), craniotomy and resection (30/92; 32.6%), and transsphenoidal resection (16/92; 17.4%). Approximately two-thirds of all operations were performed using image guidance (66/92; 71.7%) and one-third were performed using endoscopy (27/92; 29.3%). The majority of children had adjuvant therapy comprising proton beam therapy (18/59; 30.5%) or conventional radiotherapy (16/59; 27.1%). The median follow-up duration was 44 months (range 1-142 months), and approximately one-half of the children had no evidence of residual disease on MRI studies (28/59; 47.5%). Of the remaining 31 children, there was a reduction in the volume of residual disease in 8 patients (8/59; 13.6%), stable residual disease in 18 (18/59; 30.5%), and tumor growth in 5 patients (5/59; 8.5%). There was significantly reduced morbidity (p < 0.05) in all categories in the current cohort compared with our last cohort (1996-2004). The authors' institutional experience of pediatric craniopharyngioma confirms a trend toward less invasive neurosurgical procedures, most of which are now performed with the benefit of image guidance or endoscopy. Moreover, the authors have identified an expanding role for more targeted radiotherapy for children with residual disease. These advances have allowed for tumor control comparable to that achieved in previous cohorts, but with significantly reduced morbidity and mortality.

Endoscopic endonasal transsphenoidal approach for pediatric craniopharyngiomas: A case series

ObjectivesThis study aims to analyze our series of pediatric patients who underwent craniopharyngioma resection using the endoscopic endonasal transsphenoidal approach (EETA). MethodsWe collected clinical and surgical data from the charts of 20 children who underwent craniopharyngioma removal surgery using the EETA from 2007 to 2017. From the charts, we collected demographic information, results of imaging tests (size and extension of the tumor), and information regarding the surgical procedure and postoperative complications. ResultsFrom the 20 patients included in this series (12 women and eight men), 17 underwent EETA as a primary procedure, and the remaining three underwent EETA as a secondary procedure due to a relapsing tumor following previous transcranial surgery. The mean age of the patients at the time of the surgical procedure was 7.5 years (range 3–18 years). Regarding their location, 12 tumors were in the sellar and suprasellar regions, three extended into the third ventricle, and five were exclusively intrasellar. We achieved a gross total resection (GTR) of the tumor in 14 patients (70%), subtotal in five (25%), and partial in one (5%). One patient (5%) developed a cerebrospinal fluid fistula after the surgical procedure. In the postoperative follow-up period (mean time = 5.3 years; range = 2–9 years), 11 (55%) patients developed panhypopituitarism, and a relapsing tumor was later found in three (15%) patients. Regarding visual impairment, four patients had visual abnormalities preoperatively (amaurosis, n = 2; bilateral visual acuity decrease, n = 1; bilateral visual field defect, n = 1), and those did not improve or worsened postoperatively. None of the patients who did not have vision problems before the surgery developed those postoperatively. ConclusionOur results showed that the EETA is a safe and effective approach for removing craniopharyngiomas in children, as it associated with low operative morbidity and complication rates. Also, our data demonstrated that the EETA may be performed regardless of the size of the nasal cavity, pneumatization of the sphenoid sinuses, and location or extension of the tumors.

Advances in the management of craniopharyngioma in children and adults.

BackgroundChildhood and adult-onset craniopharyngioma is a rare embryogenic tumor of the sellar, suprasellar, and parasellar region. Survival rates are high; however, tumor location and treatment sequalae including endocrine deficits, visual impairment, metabolic complications, cognitive and psychosocial deficits can significantly impair patient’s quality of life. There is considerable controversy regarding the optimal management of craniopharyngiomas. Subtotal resection of the tumor followed by targeted irradiation to avoid further hypothalamic damage is currently indicated. Novel insights in the tumor’s molecular pathology present the possibility for targeted therapy possibly decreasing the rate and severity of treatment-associated morbidity.ConclusionsCraniopharyngioma should be seen as a chronic disease. To achieve optimal outcomes a multidisciplinary team of specialized neurosurgeons, neuro-radiologists, neuro-oncologists, pathologists and endocrinologists should be involved in the diagnosis, planning of the surgery, irradiation and long-term follow-up.