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Background: The treatment of craniopharyngiomas (CPs) poses challenges due to their proximity to critical neural structures, the risk of serious complications, and the impairment of quality of life after treatment. However, long-term prognostic data are still scarce. Therefore, the purpose of this retrospective study is to evaluate the long-term outcomes of patients with CPs after treatment., Material and Method: Our center retrospectively collected data on 83 children and adolescents who underwent craniopharyngioma surgery between 2001 and 2020. The medical records and radiological examination results of the patient were reviewed., Results: Outcomes were analysed for 80/83 patients who completed follow-up: 50 males (62.5%) and 30 females (37.5%), the median age at the time of diagnosis 8.4 (5.3-12.2) years. The median follow-up time was 136 (61-280) months. The 5-, 10- and 15-year overall survival (OS) rates were 100%, 98.3%, and 94.6%, respectively. Accordingly, the disease-specific survival (DSS) rates were 100%, 98.3% and 94.6%, respectively. Overall progression-free survival (PFS) rates after 5, 10 and 15 years of follow-up in the entire group were 85.4%, 72.2% and 70.1%, respectively. Multivariate analysis found that surgical resection grade was only associated with PFS outcomes [ HR = 0.031 (95% CI: 0.006, 0.163), P < 0.001], without improving OS or DSS. After undergoing recombinant human growth hormone (rhGH) replacement therapy, the total cholesterol (TC) level decreased by 0.90 mmol/L compared to baseline (P = 0.002), and the low-density lipoprotein cholesterol (LDL-C) level decreased by 0.73 mmol/L compared to baseline (P = 0.010). For liver function, compared with baseline data, aspartate aminotransferase (AST) and alanine aminotransferase (ALT) showed a downward trend, but did not reach a statistically significant level (P > 0.05)., Conclusion: Surgical treatment of CPs provides good long-time OS and DSS, even though combined with radiotherapy in only selected cases. Gross total resection (GTR) is individual positive prognostic factor. rhGH replacement could improve CPs lipid profile., Competing Interests: Declarations. Ethics approval and consent to participate: The study involving human participants has been reviewed and approved by the Ethics Committee of Peking Union Medical College Hospital (K2917), and is in compliance with the Declaration of Helsinki. After explaining the purpose of the study, written informed consent was obtained from each study participant. At the same time, the consent of the teenage participants and their legal guardians was also obtained. Competing interests: The authors declare no competing interests., (© 2024. The Author(s).)

Purpose: Craniopharyngiomas (CPs) are rare tumors of the sellar region often leading to significant comorbidities due to their close proximity to critical structures. The aim of this study was to analyze survival outcome and late toxicities after surgery and proton beam therapy (PBT) in childhood CPs., Methods and Materials: Within the prospective registry study "KiProReg" (DRKS0000536), data of 74 childhood patients with CP, receiving PBT between August 2013 to June 2022 were eligible. Late toxicities were analyzed according to the grading system of the Common Terminology Criteria for Adverse Events, version 4.0., Results: Median follow-up since first diagnosis was 4.3 years (range, 0.8-14.7). In addition, 75.7% of patients received PBT at time of disease progression or recurrence, whereas 24.3% as part of their primary therapy (definitive or adjuvant). Predominantly (85.1%), pencil beam scanning technique was used. The median total dose and initial tumor volume were 5400 cGy relative biologic effectiveness (RBE) and 17.64 cm³ (range, 3.07-300.59), respectively. The estimated (±SE) 3-year overall survival, progression-free, and cystic failure-free survival rate after PBT were 98.2% (±1.7), 94.7% (±3.0), and 76.8% (±5.4), respectively. All local failures (n = 3) were in-field relapses necessitating intervention and occurred exclusively in patients receiving PBT at progression or recurrence. Early cystic enlargements after PBT were typically asymptomatic and self-limiting. Fatigue, headaches, vision disorders, obesity, and endocrinopathies were the predominant late toxicities. No high-grade (≥3) new-onset visual impairment or cognitive deterioration occurred compared with baseline. The presence of cognitive impairments at the end of follow-up correlated with size of the planning target volume (P = .034), D mean dose to the temporal lobes (P = .032, P = .045) and the number of surgical interventions before PBT (P = .029)., Conclusions: Our findings demonstrate favorable local control rates using modern PBT with acceptable late toxicities. Cyst growth within 12 months after radiation therapy is typically not associated with tumor progression. Longer follow-up must be awaited to confirm results., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Craniopharyngioma (CP) is a rare malformational tumor characterized by high rates of recurrence and morbid obesity. However, the role of inflammatory mediators in obesity and the prognosis of patients with CP remains unknown. Therefore, the present study aimed to analyze associations of inflammatory mediators with weight-related outcomes and the prognosis of patients with CP., Methods: A total of 130 consecutive patients with CP were included in this study. The expression levels of seven inflammatory mediators and the plasma leptin concentration were investigated. Clinical parameters, weight changes, new-onset obesity, and progression-free survival (PFS) were recorded. The relationships between inflammatory mediators, clinicopathologic parameters, weight-related outcomes, and PFS were explored., Results: Compared with those in normal pituitary tissue, the expressions of inflammatory mediators in tumor tissue were higher. Higher expression levels of CXCL1 and CXCL8 were identified as independent risk factors for significant weight gain, and CXCL1 and TNF were identified as independent risk factors for new-onset postoperative obesity. Poor PFS was associated with higher expression levels of CXCL1, CXCL8, IL1A, IL6, and TNF., Conclusion: The present study revealed that inflammatory mediators are associated with morbid obesity in patients with CP. Inflammatory mediators may be the critical bridge between elevated leptin and weight-related outcomes. Additionally, PFS was associated with the expression of inflammatory mediators. Further research is needed to elucidate the underlying mechanisms of inflammatory mediators and their potential as targets for novel therapies for CP., (© 2024. The Author(s).)

Craniopharyngioma (CP) is a benign epithelial tumor predominantly localized in chiasmatic-sellar region and third ventricle. There are two histological variants of CP: adamantinoma-like (ACP, 85%) and papillomatous (PCP). These types differ significantly in origin and histological structure. PCP predominantly occurs in adults. Treatment of CP, including effectiveness of radiotherapy, is described in multiple studies and mainly devoted to pediatric patients., Objective: To clarify disease-free survival in adults with newly diagnosed CP depending on resection quality; to evaluate the effectiveness of stereotactic irradiation and treatment depending on histological characteristics of tumor., Material and Methods: We analyzed treatment outcomes in 398 adults over 10-year follow-up. Stereotactic irradiation was performed in 11.6% of patients. The follow-up data were obtained in 68.1% of patients. We compared 5-year disease-free survival rates after different resections with and without subsequent irradiation., Results: Total resection is effective and provides significantly lower risk of CP recurrence. Effectiveness of stereotactic irradiation after incomplete resection was also confirmed., Conclusion: Total resection of ACP and PCP provides high disease-free survival rates. Stereotactic irradiation after incomplete resection is followed by similar outcomes. PCPs are characterized by less aggressive growth and do not recur after total resection.

Background: Craniopharyngioma (CP) is a rare tumor in the elderly whose clinical features and prognosis are not well known in this population., Aim: To evaluate the clinicopathological features and therapeutic outcomes of CP diagnosed in the elderly., Patients and Methods: This was a retrospective, multicenter, national study of CP patients diagnosed over the age of 65 years and surgically treated., Results: From a total of 384 adult CP patients, we selected 53 (13.8%) patients (27 women [50.9%], mean age 72.3 ± 5.1 years [range 65-83 years]) diagnosed after the age of 65 years. The most common clinical symptoms were visual field defects (71.2%) followed by headache (45.3%). The maximum tumor diameter was 2.9 ± 1.1 cm. In most patients, the tumor was suprasellar (96.2%) and mixed (solid-cystic) (58.5%). The surgical approach most commonly used was transcranial surgery (52.8%), and more than half of the patients (54.7%) underwent subtotal resection (STR). Adamantinomatous CP and papillary CP were present in 51 and 45.1%, respectively, with mixed forms in the remaining. Surgery was accompanied by an improvement in visual field defects and in headaches; however, pituitary hormonal hypofunction increased, mainly at the expense of an increase in the prevalence of diabetes insipidus (DI) (from 3.9 to 69.2%). Near-total resection (NTR) was associated with a higher prevalence of DI compared with subtotal resection (87.5 vs. 53.6%, p = 0.008). Patients were followed for 46.7 ± 40.8 months. The mortality rate was 39.6% with a median survival time of 88 (95% CI: 57-118) months. DI at last visit was associated with a lower survival., Conclusion: CP diagnosed in the elderly shows a similar distribution by sex and histologic forms than that diagnosed at younger ages. At presentation, visual field alterations and headaches are the main clinical symptoms which improve substantially with surgery. However, surgery, mainly NTR, is accompanied by worsening of pituitary function, especially DI, which seems to be a predictor of mortality in this population., (© 2020 S. Karger AG, Basel.)

Craniopharyngiomas (CPs) are histologically benign tumors that are associated with high levels of morbidity. Two clinicopathological variants - adamantinomatous (ACP) and papillary (PCP) - have been described. They differ in their molecular features, whereby activating mutations in BRAF (V600E) and CTNNB1 genes characterize PCP and ACP, respectively. Recently, both variants have been shown to express elevated PD-L1 protein expression, but ACP also exhibited tumor cell-intrinsic PD-1 expression. In this study we analyze these molecular alterations in 52 cases with a long follow-up and examine their associations with immunohistochemical and clinical characteristics. ACPs comprise 73.1% of cases, while 21.2% are PCPs. Aberrant nuclear immunoreactivity for β-catenin was observed in all ACPs. BRAF p.V600E mutations were observed in 90.9% of PCPs. Only one ACP case featured both alterations. Both types of CP exhibited strong nuclear staining for p63 with diffuse and basal distribution. ACP and PCP consistently expressed PD-L1, most in a substantial percentage of tumor cells, with a distinctive spatial distribution of expression in each subtype; only ACP demonstrated PD-1 expression. There was no evidence of differences in clinical prognosis between ACPs and PCPs. The identification of hallmark molecular signatures in the two CP variants is useful for sub-categorization in routine histopathology reporting. It is also pertinent to personalized therapy and for the development of improved non-invasive therapeutic strategies in this disease.

Objective: Implication of the tumor size on oncological and functional outcomes of craniopharyngioma is inconsistently reported. The aim of this study is to assess the postoperative outcome of giant craniopharyngiomas (> 4 cm in diameter) and to elucidate the impact of tumor size on various outcome parameters and survival., Material and Methods: Forty-four patients (children aged ≤ 18 years: 25; adults: 16) with giant craniopharyngioma, operated between January 2001 and December 2015, were included in this study. Various outcomes, progression-free survival (PFS) and overall survival (OS) were calculated., Results: Gross total resection (GTR) was achieved in 17 (39%) and subtotal resection (STR) in 27 (61%) patients. Eleven patients (25%) received radiotherapy (RT) after STR. Postoperatively, new cranial nerve and motor deficits were noted in 12 (27%) and 9 (20%) patients, respectively. Tumor recurrence following GTR and STR without adjuvant RT was diagnosed in 3 (17%), and 5 (38%) patients, respectively. Following STR with RT, one (9%) experienced recurrence. PFS at 5-, and 10- year following GTR, STR, and STR + RT was 80.8%, 45.4%, and 90%, respectively. At 5- and 10- year, OS was 86.5%, 77.9% and 100% following GTR, STR, and STR + RT, respectively. The rate of GTR was significantly lower in patients with giant tumors (39% vs. 62%; Chi-square test, p value 0.008). Postoperatively, neurological deficit (20%), hypopituitarism (95%) and hypothalamic dysfunction (26%) were significantly higher for giant craniopharyngiomas. Hazards of recurrence were not significant between giant and non-giant tumors (hazard ratio 1.86; 95% CI 0.94-3.68; p 0.07). There was no significant difference in OS between the patients with giant and non-giant tumors (log-rank test 2.1; p value 0.14)., Conclusion: Tumor size should be considered as an important predictor of the postoperative functional outcome. Although the rate of GTR is less than that of small tumors, the recurrence rate, progression-free survival, and overall survival of the patients with giant tumor are comparable to non-giant tumors.

Background: Craniopharyngioma is a benign tumor that commonly develops within the suprasellar region. The tumor and treatment can have debilitating consequences for pediatric and adult patients, including vision loss and pituitary/hypothalamic dysfunction. Most craniopharyngioma series focus on treatment of the pediatric population. We evaluated the outcomes of all adult craniopharyngioma patients treated at our institution using proton therapy to report outcomes for disease control, treatment-related toxicity, and tumor response., Methods: We analyzed 14 adult patients (≥ 22 years old). All patients had gross disease at the time of radiotherapy. Five were treated for de novo disease and 9 for recurrent disease. Patients received double-scattered conformal proton therapy to a mean dose of 54 GyRBE in 1.8 GyRBE/fraction (range 52.2-54 GyRBE). Weekly magnetic resonance imaging (MRI) helped to evaluate tumor changes during radiotherapy., Results: With median clinical and radiographic follow-up of 29 and 26 months, respectively, the 3-year local control and overall survival rates were both 100%. There were no grade 3 or greater acute or late radiotherapy-related side effects. There was no radiotherapy-related vision loss or optic neuropathy. No patients required intervention or treatment replanning due to tumor changes during radiotherapy. Two patients experienced transient cyst expansion at their first post-radiotherapy MRI. Both patients were followed closely clinically and radiographically and had subsequent dramatic tumor/cyst regression, requiring no interventions., Conclusions: Our data support the safety and efficacy of proton therapy in the treatment of adult craniopharyngioma as part of primary or salvage treatment. We recommend early consideration of radiotherapy. This trial was registered at www.clinicaltrials.gov as #NCT03224767.

Purpose: Craniopharyngioma is associated with an increased risk of mortality even after surgical, radiotherapeutic and hormone supplementations. Previous studies using different designs showed a possible trend of decreasing mortality in recent years. This review summarises studies reporting standardised mortality ratio (SMR) after craniopharyngioma treatment, as well as the bias and confounding in these studies to plan further researches., Methods: PubMed and Embase was searched for manuscripts published before October 2018 using medical subject heading terms ("craniopharyngioma" or "hypopituitarism" and "mortality")., Results: Eight studies reported SMR after craniopharyngioma treatment, with a total of 2802 patients. The subgroup meta-analysis using random effects model was conducted to pool the SMR, which was 6.2 (95% CI 4.1-9.4) before 2010 and 2.9 (95% CI 2.2-3.8) after 2010 (subgroup test p < 0.01), respectively. Misclassification (one study) and selection bias (six studies) either inflated or deflated the result. The trend of increasing survival rate over the time was observed in studies without reporting SMR. Female patients, childhood-onset disease, hydrocephalus, tumour recurrence, body mass index and panhypopituitarism were identified as the important risk factors for excess mortality., Conclusions: Though bias and confounding existed across studies, the decreasing SMR and increasing survival rate over the time was in favour of a real signal. It is necessary to launch studies to further investigate the morality and risk factors after multidisciplinary treatment of craniopharyngioma in a hospital-based manner, using the modern statistical method to adjust for bias and confounding.

Background/aim: Few data are available on the utility of definitive radiation therapy (RT) for pediatric craniopharyngioma. This study sought to evaluate practice patterns and patient outcomes using the Surveillance Epidemiology and End Results database from 2004-2014., Materials and Methods: Overall survival (OS) was compared between five treatment groups, definitive radiation therapy (RT), gross total resection (GTR), subtotal resection (STR), STR+RT, and observation/biopsy only, using Kaplan-Meier analysis and log-rank tests. Multivariate Cox proportional hazards modeling determined variables independently associated with OS., Results: A total of 373 patients met the study criteria. GTR and definitive RT conferred superior OS than observation/biopsy (p=0.008 and 0.029), but were equivalent to STR+RT (p=0.350 and 0.200). GTR was associated with a higher OS than STR (p=0.027). On multivariate analysis, STR+RT, GTR, and definitive RT were associated with statistically equivalent OS (p=0.990)., Conclusion: Definitive RT for pediatric craniopharyngioma affords similar outcomes to established modalities of therapy such as GTR and STR+RT., (Copyright© 2019, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Objective: Quality of survival, prognosis and long-term outcome are often severely impaired in childhood-onset craniopharyngioma patients (CP). Identification of risk factors for sequelae such as growth hormone (GH) deficiency is important for appropriate treatment and rehabilitation., Design: In a cross-sectional study, 79 CP recruited in HIT-Endo before 2000 were analyzed according to GH substitution: a. CP never GH-treated (noGH); b. CP GH-treated only during childhood (pedGH); c. CP under GH, initiated at adulthood (adultGH); d. CP under GH during childhood and continued during adulthood (contGH)., Methods: Progression-free (PFS) and overall survival (OS), height, body mass index (BMI), psychosocial and neuropsychological status (EORTC QLQ-C30, MFI-20)., Results: OS and PFS rates were similar in all subgroups. ContGH and pedGH CP presented with increases in height (p=0.002; p=0.0001) during long-term follow-up when compared with baseline. In all subgroups except for pedGH, increases in BMI were observed when compared with BMI at diagnosis. For emotional functionality and physical fatigue, adultGH CP showed worse (p=0.037; p=0.034) response (mean: 61.4%; 12.5%) when compared with pedGH CP (mean: 83.5%; 7.7%). Observed differences were not related to irradiation and hypothalamic involvement. In terms of psychosocial status, no differences were observed between subgroups., Conclusions: We conclude that GH substitution was safe with regard to risk of tumor progression/relapse in CP. Growth was improved by GH, whereas the development of obesity was not influenced by GH substitution. However, early initiation of GH substitution after CP diagnosis might have beneficial effects on weight development and neuropsychological outcome., (© 2018 European Society of Endocrinology)

Background Craniopharyngiomas (CPs) are rare epithelial tumors arising from the Rathke's pouch remnant located along the path of the craniopharyngeal duct accounting for 1.2-4% of all primary intracranial brain tumors, the primary treatment of which is surgery. Objective Whether radical surgical resection or partial resection followed by radiotherapy is a topic of debate. We presented our 12 years single center experience on surgical resection of craniopharyngioma. Method This was a descriptive cross-sectional study conducted among forty-five patients who underwent transcranial resection of craniopharyngioma during a period of 12 years. Data were collected from medical record archives. Glassgow outcome score (GOS), electrolyte imbalance and visual complications were assessed as outcome measure. GOS > 3 was considered favorable while score ≤ 3 was considered unfavorable. Recurrence of tumors were analyzed. Result Out of 45 patients, 28 patients were male with male to female ration of 1.64. Mean age was 32.22±16.42 years. Supra-sellar craniopharyngioma were the most common location. Gross total resection was accomplished in 32 patients (71.1%) while subtotal resection among 13 patients (28.9%). Post-operative Diabetes Insipidus was developed among 35 patients (77.7%). Adamantinomatous craniopharyngioma was the most common histopathological type. Postoperative MRI with contrast was repeated to ascertain the completeness of resection. All patient with subtotal resection received radiotherapy. Follow up period ranged from 3 months to 8 years with mean of 4.2 years. Favorable outcome (GOS>3) was seen among 41 patients while unfavorable among 4 patients. Recurrence seen among 4 patients (8.9%). Overall mortality was 4 (8.8%). Conclusion Gross total excision of craniopharyngioma has a favorable outcome with acceptable morbidity.

Neutrophil-lymphocyte ratio (NLR) is a poor prognostic factor in many tumors including glioblastoma multiforme (GBM), colorectal, and prostate cancer. The aim of this study was to investigate the prognostic value of preoperative NLR in patients with craniopharyngioma.Around 149 patients of craniopharyngioma surgically were treated at the Department of Neurosurgery, West China Hospital from January 2008 to December 2010, including 84 males and 65 females aged from 6 to 70 years were retrospectively reviewed, and preoperative NLR was analyzed. Overall survival (OS), progression free survival (PFS), and quality of life (QOL) were evaluated.The 5-year OS and PFS rates were 81.21% and 75.84%. Preoperative NLR was significantly correlated with OS (HR = 1.44, 95% CI 1.16-1.79, P = .001) and PFS (HR = 1.46, 95% CI 1.22-1.74, P < .001). The best cut-off value of NLR was found to be 4 based on the receiver operator characteristics (ROC) curve. Patients with NLR ≥4 had a significantly worse QOL (P = .039), lower OS rate (P = .009), and PFS rate (P < .001).Preoperative NLR may be a simple, readily available, and valid predictor of long-term outcome in craniopharyngioma. We suggest that the NLR can provide effective guidance to neurosurgeons for more information about the tumor and prognostic evaluation.

Background: Recent studies suggest that subtotal resection (STR) followed by radiation therapy (RT) is an appealing alternative to gross total resection (GTR) for craniopharyngioma, but it remains controversial. We conducted a meta-analysis to determine whether GTR is superior to STR with RT for craniopharyngioma., Materials and Methods: A systematic search was performed for articles published until October 2017 in the PubMed, Embase, and Cochrane Central databases. The endpoints of interest are overall survival and progression-free survival. Pooled hazard ratios (HRs) and corresponding 95% confidence intervals (CIs) were calculated using a fixed or random-effects model. The data were analyzed using Review Manager 5.3 software., Results: A total of 744 patients (seven cohort studies) were enrolled for analyses. There were no significant differences between the GTR and STR with RT groups when the authors compared the pooled HRs at the end of the follow-up period. Overall survival (pooled HR = 0.76, 95% CI: 0.46-1.25, P = 0.28) and progression-free survival (pooled HR = 1.52, 95% CI: 0.42-5.44, P = 0.52) were similar between the two groups., Conclusions: The current meta-analysis suggests that GTR and STR with RT have the similar survival outcomes for craniopharyngioma., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objectives: The optimal management of craniopharyngioma is still controversial. The aim of this study is to explore microsurgical outcomes of craniopharyngioma in 335 cases., Patients and Methods: Clinical data of 335 consecutive patients with craniopharyngioma between March 2011 and March 2017 were retrospectively analyzed., Results: Gross total resection (GTR) was achieved in 265 cases (79.1%), subtotal resection (STR) was obtained in 70 cases (20.9%). The GTR rate was 81.93% in pediatric group and 78.17% in adult group respectively, no significant difference regarding the GTR rate was found in adult group compared with in pediatric group (p > 0.05). However, there was a noticeable difference in the elevated hypothalamic obesity in children group compared with in adult group after operation (p < 0.05). Multivariate analysis indicated that the tumor recurrence and surgical times played a negative role in the resection extent, the odds ratio and 95% confidence interval of the tumor recurrence and surgical times is [0.306 (0.155-0.603), (p < 0.01)] and [2.135 (1.101-4.142), (p < 0.05)] respectively. There was significant difference on panhypopituitarism between GTR and STR group (p < 0.05). However, No significant difference regarding the postoperative visual dysfunction and indepent quality of life respectively between GTR and STR group was found (p > 0.05). Additionally, there were no statistically significant differences for recurrence-free curves between GTR and STR plus adjuvant radiotherapy (p > 0.05)., Conclusions: Present findings demonstrated that tumor recurrence and surgical times contribute to negative total resection for craniopharyngioma. Postoperative precise adjuvant radiotherapy was considered in selected cases if pursuit of GTR was rather dangerous under disadvantageous removal factors., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: Surgical treatment of retrochiasmatic craniopharyngiomas is associated with higher rates of complications, mortality, failure of complete removal, and recurrence compared with craniopharyngiomas located elsewhere. These tumors lie behind the optic chiasm and when large can extend upward into the third ventricle and downward along the brain stem, making their adequate exposure challenging. Most of the proposed techniques either use a translamina terminalis route or require wide bony exposures. In this study, we assessed the feasibility of a subtemporal approach for achieving gross total resection of retrochiasmatic craniopharyngiomas., Methods: Thirty patients with retrochiasmatic craniopharyngioma underwent surgery via a subtemporal approach. The technique and surgical and preoperative and postoperative endocrinologic outcomes are described in detail., Results: Gross total resection was achieved in all cases. The average tumor volume was 7.59 mL. The average postsurgical observation time was 73.8 months. During this time, 3 recurrences were observed (10.7%). The perioperative mortality was 6.6%. The pituitary stalk was preserved in 13 cases. Partial preservation of the pituitary stalk did not offer any advantage in terms of pituitary function. No postoperative vision worsening or new fixed neurologic deficits were observed. Among the 22 patients with preoperative vision impairment, 18 reported a significant improvement. The most common abnormalities within the temporal lobe on the side of the exposure seen on control magnetic resonance imaging were mild temporal horn enlargement (13 cases) and T2 hyperintensity (7 cases)., Conclusions: A subtemporal approach can be an attractive alternative approach to accessing retrochiasmatic craniopharyngiomas. Outcomes are comparable to those associated with other widely used and time-consuming exposures., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective: Most studies in patients with craniopharyngioma did not investigate morbidity and mortality relative to the general population nor evaluated risk factors for excess morbidity and mortality. Therefore, the objective of this study was to examine excess morbidity and mortality, as well as their determinants in patients with craniopharyngioma., Design: Hospital-based retrospective cohort study conducted between 1987 and 2014., Methods: We included 144 Dutch and 80 Swedish patients with craniopharyngioma identified by a computer-based search in the medical records (105 females (47%), 112 patients with childhood-onset craniopharyngioma (50%), 3153 person-years of follow-up). Excess morbidity and mortality were analysed using standardized incidence and mortality ratios (SIRs and SMRs). Risk factors were evaluated univariably by comparing SIRs and SMRs between non-overlapping subgroups., Results: Patients with craniopharyngioma experienced excess morbidity due to type 2 diabetes mellitus (T2DM) (SIR: 4.4, 95% confidence interval (CI): 2.8-6.8) and cerebral infarction (SIR: 4.9, 95% CI: 3.1-8.0) compared to the general population. Risks for malignant neoplasms, myocardial infarctions and fractures were not increased. Patients with craniopharyngioma also had excessive total mortality (SMR: 2.7, 95% CI: 2.0-3.8), and mortality due to circulatory (SMR: 2.3, 95% CI: 1.1-4.5) and respiratory (SMR: 6.0, 95% CI: 2.5-14.5) diseases. Female sex, childhood-onset craniopharyngioma, hydrocephalus and tumour recurrence were identified as risk factors for excess T2DM, cerebral infarction and total mortality., Conclusions: Patients with craniopharyngioma are at an increased risk for T2DM, cerebral infarction, total mortality and mortality due to circulatory and respiratory diseases. Female sex, childhood-onset craniopharyngioma, hydrocephalus and tumour recurrence are important risk factors., (© 2018 European Society of Endocrinology.)

Purpose: Craniopharyngioma is associated with metabolic alterations leading to increased cardiovascular mortality. Recently, the visceral adiposity index has been proposed as a marker of visceral adipose tissue dysfunction and of the related cardiometabolic risk. The role of the visceral adiposity index has never been explored in craniopharyngioma patients. We assessed the cardiometabolic risk on the basis of the visceral adiposity index in craniopharyngioma patients., Methods: We evaluated data of 24 patients treated for craniopharyngioma in a single-centre. We investigated the relationship among patients' clinical and biochemical features, cardiovascular risk -assessed by the Framingham and the atherosclerotic cardiovascular disease risk scores-, visceral adiposity index and adipose tissue dysfunction severity., Results: Increased visceral adiposity index was found in 8 patients (33%). Adipose tissue dysfunction resulted to be severe, moderate or mild in 5, 2 and 1 cases. Increased visceral adiposity index significantly correlated with the occurrence of metabolic syndrome (p 0.027), IRI (p 0.001), triglycerides (p < 0.001), HOMA-IR (p < 0.001) and with lower ISI-Matsuda (p 0.005) and HDL-cholesterol (p < 0.001). Higher degree of adipose tissue dysfunction associated with increased insulin resistance. No gender difference was found for visceral adiposity index, adipose tissue dysfunction severity, and cardiovascular risk scores. Patients with adulthood onset craniopharyngioma showed higher Framingham risk score (p 0.004), atherosclerotic cardiovascular disease 10-year (p < 0.001) and lifetime (p 0.018) risk scores than those with childhood onset disease., Conclusions: Visceral adiposity index is increased in one third of our patients with craniopharyngioma, even if metabolic syndrome does not occur. Increased visceral adiposity index and adipose tissue dysfunction severity correlate with insulin sensitivity parameters, do not correlate with Framingham or atherosclerotic cardiovascular disease risk scores, and are not influenced by gender and age of disease onset.

Purpose: Hypopituitarism is a rare disorder with significant morbidity. To study the evidence on the association of premature mortality and hypopituitarism., Methods: A comprehensive search of multiple databases: MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews, and Scopus was conducted through August, 2015. Eligible studies that evaluated patients with hypopituitarism and reported mortality estimates were selected following a predefined protocol. Reviewers, independently and in duplicate, extracted data and assessed the risk of bias., Results: We included 12 studies (published 1996-2015) that reported on 23,515 patients. Compared to the general population, hypopituitarism was associated with an overall excess mortality (weighted SMR of 1.55; 95 % CI 1.14-2.11), I 2  = 97.8 %, P = 0.000. Risk factors for increased mortality included younger age at diagnosis, female gender, diagnosis of craniopharyngioma, radiation therapy, transcranial surgery, diabetes insipidus and hypogonadism., Conclusion: Hypopituitarism may be associated with premature mortality in adults. Risk is particularly higher in women and those diagnosed at a younger age.

Craniopharyngiomas are benign epithelial tumors having a dysembryogenetic origin, which are usually located in the sellar/parasellar and/or third ventricle regions. Gross total resection is the modern standard of treatment for these tumors because of a low recurrence rate. However, this surgery in some patients with craniopharyngioma often leads to the development or worsening of diencephalic disorders poorly responding to treatment. Perhaps, in these cases, subtotal or partial tumor resection or implantation of an Ommaya reservoir into the tumor cyst followed by stereotactic radiotherapy/radiosurgery may provide better functional outcomes and higher life quality in patients, with tumor growth control being reasonable (according to the published data, the mean 10-year disease-free survival is 66.9% after total tumor resection and 79.6% after combined treatment). The paper presents a review of the literature on radiological treatment of craniopharyngiomas. We discuss the issues of indications, optimal timing of radiotherapy/radiosurgery, its efficacy, and treatment outcomes in terms of complications and quality of life. Particular attention is paid to enlargement of craniopharyngioma cysts during and after radiological treatment.

Objective: The endoscopic extended transsphenoidal approach for suprasellar craniopharyngiomas may be a really alternative to the transcranial approach in many cases. The authors present their experience with this technique in 136 patients with craniopharyngiomas., Methods: From the past 7 years 204 patients with different purely supradiaphragmatic tumors underwent removal by extended endoscopic transsphenoidal transtuberculum transplanum approach. Most of the patients (136) had craniopharyngiomas (suprasellar, intra-extraventricular). The patients were analyzed according to age, sex, tumor size, growth and tumor structure, and clinical symptoms. Twenty-five patients had undergone a previous surgery. The mean follow-up was 42 months (range, 4-120 months). The operation is always performed with the bilateral endoscopic endonasal anterior extended transsphenoidal approach., Results: A gross-total removal was completed in 72%. Improvement of vision or absence of visual deterioration after operation was observed in 89% of patients; 11% had worsening vision after surgery. Endocrine dysfunction did not improve after surgery, new hypotalamopituitary dysfunction (anterior pituitary dysfunction or diabetes insipidus) or worsening of it was observed in 42.6%. Other main complications included transient new mental disorder in 11%, temporary neurological postoperative deficits in 3.7%, bacterial meningitis in 16%, cerebrospinal fluid leaks in 8.8%. The recurrence rate was 20% and the lethality was 5.8%., Conclusions: Resection of suprasellar craniopharyngiomas using the extended endoscopic approach is a more effective and less traumatic technology, able to provide resection of the tumor along with high quality of life after surgery, and relatively rare postoperative complications and mortality., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objective: Craniopharyngioma resection is one of the most challenging surgical procedures. Herein, we describe our extended endoscopic endonasal transsphenoidal surgery (EETS) technique, and the results of 9 years of use on primary and recurrent/residual craniopharyngiomas., Methods: This study reviewed 28 EETSs in 25 patients with craniopharyngiomas between January 2006 and September 2015. The patients were divided into 2 groups, newly diagnosed patients (group A, n = 15), and patients having residual or recurrent tumors (group B, n = 10). There was no significant difference between the groups in terms of the largest tumor diameter (P = 0.495), and all patients underwent EETS. The clinical and ophthalmologic examinations, imaging studies, endocrinologic studies, and operative findings for these cases were reviewed retrospectively., Results: The number of gross total resections in group A was 13/15, and 7/10 in group B. Three of the patients developed postoperative cerebrospinal fluid leakage (all in group A). There were no neurovascular or ophthalmologic complications, and no meningitis or mortality was observed., Conclusions: There has been a notable increase in the use of EETS in the treatment of craniopharyngiomas during the last decade. Despite its increased use in the treatment of primary craniopharyngiomas, its implementation for recurrent or residual craniopharyngiomas has been viewed with suspicion. In this study, the results have been presented separately for primary and recurrent/residual craniopharyngiomas, so that the results can be compared. Overall, EETS is a reliable and successful surgical treatment method for primary and recurrent/residual craniopharyngiomas., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Quality of life of craniopharyngioma patients can be severely impaired by derangement of hypothalamic function. A classification, taking into account preoperative hypothalamic damage, evaluated by magnetic resonance imaging (MRI), and correlating it with postoperative weight change is still missing in the literature. The aim of our study is to identify objective radiological criteria as preoperative prognostic factors for hypothalamic damage. Pre- and post-operative MRI and clinical data of 47 patients, treated at our Institution for craniopharyngioma, were retrospectively analyzed, based on radiological variables, identified as prognostic factor for hypothalamic involvement. Main factors associated with postoperative obesity were hypothalamic hyperintensity in T2-weighted/FLAIR imaging (p < 0.033), mammillary body involvement according to Müller classification (p < 0.020), unidentifiable pituitary stalk (p < 0.001), dislocated chiasm (p < 0.038), either not visible infundibular recess (p < 0.019) or unrecognizable supra-optic recess (p < 0.004), and retrochiasmatic tumor extension (p < 0.019). Accordingly, postoperative hypothalamic syndrome was associated with peritumoral edema in T2-weighted/FLAIR images (p < 0.003), unidentifiable hypothalamus (p < 0.024), hypothalamic compression (p < 0.006), fornix displacement (p < 0.032), and unrecognizable supra-optic recess (p < 0.031). Ultimately, variables identified as predictive factors of postoperative hypothalamic syndrome were the degree of hypothalamic involvement according to the classification described by Sainte-Rose and Puget (p < 0.002; grade 0 vs 2 p < 0.001), Van Gompel (p < 0.002; grade 0 vs 1, p < 0.027; and grade 0 vs 2, p < 0.002), and Muller (p < 0.006; grade 0 vs 1, p < 0.05; and grade 0 vs 2, p < 0.004). The identification of these predictive factors will help to define and score the preoperative hypothalamic involvement in craniopharyngioma patients.

The objective of this study is to investigate β-catenin expression in craniopharyngioma patients and determine its significance in predicting the prognosis of this disease. Fifty craniopharyngioma patients were enrolled in this study. Expression of β-catenin in tumor specimens collected from these patients was examined through immunostaining. In addition, mutation of exon 3 in the β-catenin gene, CTNNB1, was analyzed using polymerase chain reaction, denaturing high-pressure liquid chromatography, and DNA sequencing. Based on these results, we explored the association between membranous β-catenin expression, clinical and pathologic characteristics, and prognoses in these patients. Of all craniopharyngioma specimens, 31 (62.0%) had preserved membranous β-catenin expression, whereas the remaining 19 specimens (38.0%) displayed aberrant expression. Statistical analysis showed a significant correlation between aberrant membranous β-catenin expression and CTNNB1 exon 3 mutation, as well as between aberrant membranous β-catenin expression and the histopathologic type of craniopharyngioma and type of resection in our patient population. Furthermore, aberrant membranous β-catenin expression was found to be associated with poor patient survival. Results of Kaplan-Meier survival analysis and Cox regression analysis further confirmed this finding. In conclusion, our study demonstrated that aberrant membranous β-catenin expression was significantly correlated with poor survival in patients with craniopharyngioma. This raises the possibility for use of aberrant membranous β-catenin expression as an independent risk factor in predicting the prognosis of this disease., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: Quality of life (QoL) and long-term prognosis are frequently, and often severely, impaired in craniopharyngioma (CP) patients. Knowledge of risk factors for long-term outcome is important for optimization of treatment., Methods: Overall survival (OS) and progression-free survival (PFS), body mass index (BMI), neuropsychological status (EORTCQLQ-C30, MFI-20), and psychosocial status were analyzed in 261 patients with childhood-onset CP diagnosed before 2000 and longitudinally observed in HIT-Endo., Results: Twenty-year OS was lower (P = .006) in CP with hypothalamic involvement (HI) (n = 132; 0.84 ± 0.04) when compared with CP without HI (n = 82; 0.95 ± 0.04). OS was not related to degree of resection, sex, age at diagnosis, or year of diagnosis (before/after 1990). PFS (n = 168; 0.58 ± 0.05) was lower in younger patients (<5 y at diagnosis) (n = 30; 0.39 ± 0.10) compared with patients aged 5-10 years (n = 66; 0.52 ± 0.08) and older than 10 years (n = 72; 0.77 ± 0.06) at diagnosis. PFS was not associated with HI, degree of resection, or sex. HI led to severe weight gain during the first 8-12 years of follow-up (median BMI increase: +4.59SD) compared with no HI (median increase: +1.20SD) (P = .00). During >12 years of follow-up, patients with HI presented no further increase in BMI. QoL in CP patients with HI was impaired by obesity, physical fatigue, reduced motivation, dyspnea, diarrhea, and nonoptimal psychosocial development., Conclusions: OS and QoL are impaired by HI in long-term survivors of CP. HI is associated with severe obesity, which plateaus after 12 years. OS/PFS are not related to degree of resection, but gross-total resection should be avoided in cases of HI to prevent further hypothalamic damage, which exacerbates sequelae., (© The Author(s) 2015. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Object: In this paper the authors present their experience treating children with recurrent craniopharyngioma who were initially managed with surgery followed by conformal radiation therapy (CRT)., Methods: A departmental oncology information system was queried to identify all children (< 18 years old) who received CRT for a craniopharyngioma between 1998 and 2010 (inclusive) and specifically those who experienced tumor progression. For each patient, the authors recorded the type of recurrence (solid, cystic, or both), the time interval to first progression and each subsequent progression, the associated treatment complications, and disease status at last follow-up evaluation., Results: Among the 97 patients that met criteria for entry into this study, 18 (18.6%) experienced tumor progression (9 cystic, 3 solid, 6 cystic and solid). The median time to first recurrence was 4.62 years (range 1.81-9.11 years). The subgroup included 6 female and 12 male patients with a median age of 7.54 years (range 3.61-13.83 years). Ten patients experienced first progression within 5 years of CRT. The 5- and 10-year treatment-free survival rates for the entire cohort were 89.0% (95% confidence interval [CI] 80.5%-93.9%) and 76.2% (95% CI 64%-85%), respectively. Seven patients had a single episode of progression and 11 had more than 1. The time interval between each subsequent progression was progressively shorter. The 18 patients underwent 38 procedures. The median follow-up duration for this group was 9.32 years (range 4.04-19.0 years). Three patients died, including 1 from perioperative complications., Conclusions: Craniopharyngioma progression after prior irradiation is exceedingly difficult to treat and local control is challenging despite repeated surgical procedures. Given our results, gross-total resection may need to be the surgical goal at the time of first recurrence, if possible. Decompressing new cyst formation alone has a low rate of long-term success.

Context: Craniopharyngiomas (CPs) in adults have been associated with excess mortality., Objective: The aim of the study was to investigate mortality and morbidity in patients with childhood-onset and adult-onset CP., Methods: PATIENTS with CP were identified and followed in Swedish national health registries, 1987 through 2011. The inclusion criteria for the CP diagnosis were internally validated against patient records in 28% of the study population., Settings: This was a nationwide population-based study., Patients: A total of 307 patients (151 men and 156 women) were identified and included (mean follow-up, 9 years; range, 0-25 years). The inclusion criteria had a positive predictive value of 97% and a sensitivity of 92%., Intervention: There were no interventions., Main Outcome Measures: Standardized mortality ratios (SMRs) and standardized incidence ratios (SIRs) with 95% confidence intervals were calculated using the Swedish population as the reference., Results: During the study, 54 patients died compared with the expected number of 14.1, resulting in an SMR of 3.2 (2.2-4.7) for men and 4.9 (3.2-7.2) for women. PATIENTS with childhood-onset (n = 106) and adult-onset (n = 201) CP had SMRs of 17 (6.3-37) and 3.5 (2.6-4.6), respectively. PATIENTS with hypopituitarism (n = 250), diabetes insipidus (n = 110), and neither of these (n = 54) had SMRs of 4.3 (3.1-5.8), 6.1 (3.5-9.7), and 2.7 (1.4-4.6), respectively. The SMR due to cerebrovascular diseases was 5.1 (1.7-12). SIRs were 5.6 (3.8-8.0) for type 2 diabetes mellitus, 7.1 (5.0-9.9) for cerebral infarction, 0.7 (0.2-1.7) for myocardial infarction, 2.1 (1.4-3.0) for fracture, and 5.9 (3.4-9.4) for severe infection. The SIR for all malignant tumors was 1.3 (0.8-2.1)., Conclusions: This first nationwide population-based study of patients with CP demonstrated excess mortality that was especially marked in patients with childhood-onset disease and among women. Death due to cerebrovascular diseases was increased 5-fold. Hypopituitarism and diabetes insipidus were negative prognostic factors for mortality and morbidity. PATIENTS with CP had increased disease burden related to type 2 diabetes mellitus, cerebral infarction, fracture, and severe infection.

Objective: Pure intraventricular craniopharyngioma is a rare subtype of craniopharyngioma that attaches frequently to the hypothalamus. The main challenge in tumor removal is protection of hypothalamic structure. The aim of our study was to set up a feasible risk-evaluation approach to help the surgeons make an individual treatment plan., Methods: We reviewed retrospectively 24 patients with pure intraventricular craniopharyngioma who underwent surgical therapy. Third ventricular deformation and thalamic attachment of the tumor were assessed by preoperative magnetic resonance imaging and intraoperative inspection. Correlations between the outcome, extent of removal, and different attachment grades were analyzed., Results: Preoperative magnetic resonance imaging grade had a significant correlation with functional outcome. Attachment grade was significantly correlated with endocrine, functional, and radiologic outcome. In the gross total removal (GTR) group, significantly more newly developed endocrinologic deficits were observed compared with patients who underwent subtotal removal (STR). All 6 GTR cases with the greatest attachment grade (grade 2) acquired newly developed endocrine insufficiency., Conclusions: Attachment and deformation grade evaluated based on our criteria are significantly correlated with postoperative outcome. GTR of tightly attached tumor is associated with worse endocrinologic, functional, and radiologic outcomes compared with STR. Hence, we suggest that individual surgical plans should be made according to the grade of tumor attachment and hypothalamic deformation. STR should used in tumors with a high grade to achieve a good long-term outcome and avoid severe postoperative sequelae., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Object: Although craniopharyngiomas are benign intracranial tumors, their high recurrence rates and intimate associations with surrounding neurovascular structures make gross tumor resection challenging. Stereotactic radiosurgery has been introduced as a valuable adjuvant therapy for recurrent or residual craniopharyngiomas. However, studies with large patient populations documenting long-term survival and progression-free survival rates are rare in the literature. The current study aims to report the long-term radiosurgical results and to define the prognostic factors in a large cohort of patients with a craniopharyngioma., Methods: A total of 137 consecutive patients who underwent 162 sessions of Gamma Knife surgery (GKS) treatments at the Taipei Veterans General Hospital between 1993 and 2012 were analyzed. The patients' median age was 30.1 years (range 1.5-84.9 years), and the median tumor volume was 5.5 ml (range 0.2-28.4 ml). There were 23 solid (16.8%), 23 cystic (16.8%), and 91 mixed solid and cystic (66.4%) craniopharyngiomas. GKS was indicated for residual or recurrent craniopharyngiomas. The median radiation dose was 12 Gy (range 9.5-16.0 Gy) at a median isodose line of 55% (range 50%-78%)., Results: At a median imaging follow-up of 45.7 months after GKS, the rates of tumor control were 72.7%, 73.9%, and 66.3% for the solid, cystic, and mixed tumors, respectively. The actuarial progression-free survival rates plotted by the Kaplan-Meier method were 70.0% and 43.8% at 5 and 10 years after radiosurgery, respectively. After repeated GKS, the actuarial progression-free survival rates were increased to 77.3% and 61.2% at 5 and 10 years, respectively. The overall survival rates were 91.5% and 83.9% at the 5- and 10-year follow-ups, respectively. Successful GKS treatment can be predicted by tumor volume (p = 0.011). Among the 137 patients who had clinical follow-up, new-onset or worsened pituitary deficiencies were detected in 11 patients (8.0%). Two patients without tumor growth had a worsened visual field, and 1 patient had a new onset of third cranial nerve palsy., Conclusions: The current study suggests that GKS is a relatively safe modality for the treatment of recurrent or residual craniopharyngiomas, and it is associated with improved tumor control and reduced in-field recurrence rates. Acceptable rates of complications occurred.

Purpose: We compared proton beam therapy (PBT) with intensity modulated radiation therapy (IMRT) for pediatric craniopharyngioma in terms of disease control, cyst dynamics, and toxicity., Methods and Materials: We reviewed records from 52 children treated with PBT (n=21) or IMRT (n=31) at 2 institutions from 1996-2012. Endpoints were overall survival (OS), disease control, cyst dynamics, and toxicity., Results: At 59.6 months' median follow-up (PBT 33 mo vs IMRT 106 mo; P<.001), the 3-year outcomes were 96% for OS, 95% for nodular failure-free survival and 76% for cystic failure-free survival. Neither OS nor disease control differed between treatment groups (OS P=.742; nodular failure-free survival P=.546; cystic failure-free survival P=.994). During therapy, 40% of patients had cyst growth (20% requiring intervention); immediately after therapy, 17 patients (33%) had cyst growth (transient in 14), more commonly in the IMRT group (42% vs 19% PBT; P=.082); and 27% experienced late cyst growth (32% IMRT, 19% PBT; P=.353), with intervention required in 40%. Toxicity did not differ between groups. On multivariate analysis, cyst growth was related to visual and hypothalamic toxicity (P=.009 and .04, respectively). Patients given radiation as salvage therapy (for recurrence) rather than adjuvant therapy had higher rates of visual and endocrine (P=.017 and .024, respectively) dysfunction., Conclusions: Survival and disease-control outcomes were equivalent for PBT and IMRT. Cyst growth is common, unpredictable, and should be followed during and after therapy, because it contributes to late toxicity. Delaying radiation therapy until recurrence may result in worse visual and endocrine function., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: Craniopharyngiomas are successfully managed with surgery and/or adjuvant chemoradiotherapy but have been documented to undergo malignant transformation (MT), albeit very rarely, with only 23 reported cases. The exact cause and pathogenesis of this MT are unknown, although the literature has suggested a possible correlation with radiotherapy., Objective: To review the reported cases of malignancy, in particular looking at the incidence, tumor characteristics, previous treatment modalities, and median survival., Methods: We conducted a PUBMED, SCOPUS, OVID SP, and INFORMA search with a combination of key words: craniopharyngioma, malignancy, transformation, neoplasm, radiation therapy, and anaplastic. We identified 23 cases relevant to our study., Results: Median age at the time of diagnosis of malignant craniopharyngiomas was 31 years (range, 10-66 years); 52.6% of the patients were female. Histologically, the most common tumor types were squamous cell carcinoma (80.96%), with adamantinomatous cell type being the most common morphology (89.47%). We found that 21.7% of the cases were diagnosed as malignant craniopharyngioma at first biopsy. Of the rest, the median time from initial benign diagnosis to MT was 8.5 years (range, 3-55 years). Median overall survival after MT was 6 months (range, 2 weeks-5 years). Using the Spearman rank correlation, we found no correlation between the use of radiation therapy (correlation coefficient, -0.25; P < .05) or its dosage (correlation coefficient, -0.26; P < .05) and MT., Conclusion: Malignant craniopharyngiomas are rare and are associated with a poor prognosis. MTs occur years after the initial benign craniopharyngioma diagnosis and are associated with multiple benign craniopharyngioma recurrence. Results also show that, contrary to widespread belief, there is a poor correlation between radiotherapy and MT.

Objective: To evaluate clinical outcome in a 10-year consecutive series of children operated for sellar and parasellar tumors with special focus on neuropsychology and endocrinology., Patients and Methods: We analyzed 61 children (30 female) under 18 years of age (mean age 9.9, range 1 month-17 years) operated between 2000 and 2010. Medical records were evaluated retrospectively; postoperative histologic diagnoses included 20 craniopharyngiomas, 17 gliomas, 6 pituitary adenomas and 18 rare tumor entities., Results: Of 61 patients, 58 (95%) were still alive at last follow-up. Three patients died, all due to progression of malignant rhabdoid tumors. Postoperative clinical morbidity consisted of endocrinological (66%), visual (60.7%) and other neurological deficits (55.9%) after a mean follow-up of 44 months. When compared to all other tumor entities in this series, craniopharyngiomas were associated with high rates of gross-total resection (p=0.008), frequent progression of residual tumor (p=0.005) scotomas (p=0.013), persistent diabetes insipidus (p<0.001), and panhypopituitarism (p<0.001). Surgically treated gliomas showed higher rates of motor weakness (p=0.004), double vision (p<0.001), and milder forms of endocrinopathy (single hormone deficits, p=0.02). In general, deterioration in school performance was associated with multiple surgeries (p=0.018) and radiotherapy (p=0.021)., Conclusion: Excellent overall survival in these patients is possible, however malignant rhabdoid tumors have a poor prognosis. Aggressive treatment is associated with significant morbidity. Children operated for craniopharyngioma showed an expected high rate of endocrine deterioration, whereas glioma patients had higher incidences of motor weakness and double vision. The treating physicians should be well aware of all these considerable postoperative deficits, especially when facing interdisciplinary management decisions, and for the informed consent discussions with the patient and the parents., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Background: Prognosis in childhood cranio-pharyngioma, is frequently impaired due to sequelae. Radical surgery was the treatment of choice for decades. Even at experienced facilities radical surgery can result in hypothalamic disorders such as severe obesity., Objective: We analyzed, whether treatment strategies for childhood craniopharyngioma patients recruited in GPOH studies have changed during the last 12 years., Materials and Methods: We compared the grade of pre-surgical hypothalamic involvement, treatment, degree of resection and grade of surgical hypothalamic lesions between patients recruited in KRANIOPHARYNGEOM 2000 (n=120; 2001-2007) and KRANIOPHARYNGEOM 2007 (n=106; 2007-2012)., Results: The grade of initial hypothalamic involvement was similar in patients treated 2001-2007 and 2007-2012. The realized treatment was more radical (p=0.01) in patients recruited 2001-2007 (38%) when compared with patients treated 2007-2012 (18%). In patients with pre-surgical involvement of anterior/posterior hypothalamic areas, the rate of hypothalamus-sparing operations resulting in no (further) hypothalamic lesions was higher (p=0.005) in patients treated 2007-2012 (35%) in comparison with the 2001-2007 cohort (13%). Event-free-survival rates were similar in both cohorts., Conclusions: A trend towards less radical surgical approaches is observed, which was accompanied by a reduced rate of severe hypothalamic lesions. Radical surgery is not an appropriate treatment strategy in patients with hypothalamic involvement. Despite previous recommendations to centralize treatment at specialized centers, a trend towards further decentralization was seen., (© Georg Thieme Verlag KG Stuttgart · New York.)

Purpose: We report long-term outcomes and complications of craniopharyngioma patients referred to our institution., Methods and Materials: Between 1971 and 2010, 123 consecutive patients received primary treatment for craniopharyngioma in British Columbia and were referred to our institution. The median age was 30 years (range, 2-80 years). Thirty-nine percent of patients were treated primarily with subtotal resection (STR) and radiation therapy (RT), 28% with STR alone, 15% with gross total resection, 11% with cyst drainage (CD) alone, 5% with CD+RT, and 2% with RT alone. Eight percent of patients received intracystic bleomycin (ICB) therapy., Results: Median follow-up was 8.9 years, and study endpoints were reported at 10 years. Ten-year Kaplan-Meier progression-free survival (PFS) was 46%. Patients treated with STR+RT or CD+RT had the highest PFS (82% and 83%, respectively). There were no significant differences between PFS after adjuvant versus salvage RT (84% vs 74%, respectively; P=.6). Disease-specific survival (DSS) was 88%, and overall survival (OS) was 80%. Primary treatment modality did not affect DSS or OS, while older age was a negative prognostic factor for OS but not DSS. Kaplan-Meier rates for visual deterioration, anterior pituitary hormone deficiency, diabetes insipidus, seizure disorder, and cerebrovascular events (CVE) due to treatment, not tumor progression, were 27%, 76%, 45%, 16%, and 11%, respectively. The CVE rate was 29% in patients who received ICB compared to 10% in those who did not (P=.07)., Conclusions: We report favorable PFS in patients with craniopharyngioma, especially in those who received RT after surgery. DSS and OS rates were excellent regardless of primary treatment modality. We observed a high incidence of hypopituitarism, visual deterioration, and seizure disorder. Eleven percent of patients experienced CVEs after treatment. There was a suggestion of increased CVE risk in patients treated with ICB., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Craniopharyngiomas are embryogenic malformations of the sellar region. With an overall incidence of 0.5-2.0 new cases per million population per year, approximately 30-50% of all cases represent childhood craniopharyngioma. Therapy of choice in patients with favorable tumor localization is complete resection with a specific focus on maintaining functions of the optical nerve and hypothalamic-pituitary axes. In patients whose unfavorable tumor localization makes maintaining hypothalamic functionality surgically challenging, a limited resection followed by local irradiation is recommended. The overall survival rates are high (92%) but relapses and reduced QoL in survivors are also frequent. Because irradiation is efficient in preventing tumor progression, appropriate timing of post-surgical irradiation is currently under investigation in a randomized trial (KRANIOPHARYNGEOM 2007). Childhood craniopharyngioma should be recognized as a chronic disease requiring constant monitoring of the clinical and QoL consequences in order to provide optimal care of surviving patients.

Aim: Treatment of craniopharyngiomas remains challenging. The aim of this study was to evaluate results of initial surgical management of craniopharyngiomas via the extended pterional approach., Material and Methods: Nineteen patients (10 female; median age of 23 years; ranging from 10 months to 67 years) undergone initial surgery for craniopharyngiomas through the extended pterional approach. Data were retrospectively collected by reviewing medical records, operative reports and imaging results., Results: The median size of craniopharyngiomas was 3.2 cm (range 1.3 - 4.8). Visual deterioration (n=12) and headache (n=10) were the most common symptoms on admission. After surgery, one patient died due to medical complications, 12 patients developed diabetes insipidus and eight patients developed anterior pituitary dysfunction. Median follow-up period was 9 years (range 2 - 13). Patients with progression of craniopharyngioma (n=5) when compared to patients without progression of craniopharyngioma (n=14) were significantly younger at the time of surgery, had less visual deterioration before surgery and had higher rates of not changed vision after surgery. Children had larger craniopharyngiomas when compared to adults., Conclusion: Initial surgery for craniopharyngioma via the extended pterional approach is a safe and effective treatment option with low postoperative mortality, and acceptable postoperative morbidity and recurrence rate.

Background: Although craniopharyngiomas are considered "benign" neoplasms by the World Health Organization classification, these tumors may create significant morbidity and mortality in patients. Hypothalamic obesity is a frequent complication of craniopharyngiomas and is refractory to current management options., Patients/methods: We reviewed 24 cases of craniopharyngiomas treated from 1992 to 2010 in patients <18 years of age regarding clinical presentation, neuroimaging, recurrence, morbidity, and mortality, with particular attention to hypothalamic obesity., Results: Our cohort conformed to published data in regard to neuroimaging characteristics, and clinical findings in the areas of endocrine, visual, neurological, neurobehavioral, and hypothalamic domains. At last follow-up, 53% of our patients were overweight (8%) or obese (46%). Only 25% of our patients had a healthy body mass index. Contrasting these data with body mass indices at diagnosis, where 21% of patients were overweight and 17% were obese, we found that there was a significant trend towards obesity over time. A significant portion of our mortality appears to be related to complications of obesity. The Native American population in Arizona appears to have a statistically greater incidence of obesity in childhood. Despite our small sample size, 75% of our Native Americans were obese at last follow-up and accounted for 50% of the mortality., Conclusion: Hypothalamic obesity is a significant complication of craniopharyngiomas associated with increased mortality. The development of hypothalamic obesity is influenced by premorbid obesity, genetics, and therapy received, specifically radiation. Because of the intractability of hypothalamic obesity, improved understanding of neuroendocrine mechanisms, genomics, and newer antiobesity medications will be necessary to curb this significant complication., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Context: Hypothalamic damage caused by craniopharyngioma (CP) is associated with poor functional outcome., Objective: To assess cognitive function and quality of life in childhood-onset CP on hormonal replacement, including GH treatment., Design: A cross-sectional study with a median follow-up time of 20 years (1-40)., Setting: Patients were recruited from the South Medical Region of Sweden., Participants: The study included 42 patients (20 women) surgically treated for a childhood-onset CP between 1958 and 2000. Patients were aged ≥17 years. Equally many controls, matched for age, sex, residence, and smoking habits, were included. Tumor growth into the third ventricle was found in 25 patients., Main Outcome Measures: All subjects were examined with a battery of cognitive tests and the following questionnaires: Symptom Checklist-90, the Interview Schedule for Social Interaction, and the Social Network concept., Results: The CP patients had lower cognitive performance, reaching statistical significance in 12 of 20 test variables, including executive function and memory. Comparison of patients with tumor growth into the third ventricle to controls revealed a significant lower mean total score (P = .006). A significant negative correlation was recorded between mean z-score of cognitive performance and years since operation (r = -0.407; P = .014). No statistically significant group differences were observed across any of the 9 Symptom Checklist-90 subscales., Conclusions: Adults with childhood-onset CP, on hormone replacement, including GH treatment, have memory defects, disturbed attention, and impaired processing speed. Patients with hypothalamic involvement are more affected. Patients rated their quality of life as good as their matched controls.

Background: Craniopharyngioma tumors and their treatment can lead to significant long-term morbidity due to their proximity to vital structures. The optimal treatment has been debated for many years. We aimed to review the long-term outcomes of children treated for craniopharyngioma in our institution over the past decade and describe trends in treatment and outcomes over the past 3 decades., Methods: Charts of children with craniopharyngioma treated and followed at The Hospital for Sick Children between 2001 and 2011 were reviewed. Data regarding findings at diagnosis, treatment, and long-term outcomes were analyzed. Comparison was made with previously published data from our institution., Results: Data from 33 patients are included; mean age at treatment, 10.7 ± 4.8 years. In 18 children (55%), the initial surgical approach was tumor cyst decompression with or without adjuvant therapy, compared with only 0-2% in the preceding decades (P < .01). Diabetes insipidus occurred in 55% of children and panhypopituitarism in 58% compared with 88% (P < .01) and 86% (P < .01), respectively, in the previous 10 years. Overall, there was a 36% reduction in the number of children who developed severe obesity compared with the preceding decade. Body mass index at follow-up was associated with body mass index at diagnosis (P = .004) and tumor resection as an initial treatment approach (P = .028)., Conclusions: A shift in surgical treatment approach away from gross total resection has led to improved endocrine outcomes. This may have beneficial implications for quality of life in survivors.

A craniopharyngioma (CP) is an embryonic malformation of the sellar and parasellar region. The annual incidence is 0.5-2.0 cases/million/year and approximately 60 % of CP are seen in adulthood. Craniopharyngiomas have the highest mortality of all pituitary tumors. Typical initial manifestations at diagnosis in adults are visual disturbances, hypopituitarism and symptoms of elevated intracranial pressure. The long-term morbidity is substantial with hypopituitarism, increased cardiovascular risk, hypothalamic damage, visual and neurological deficits, reduced bone health, and reduction in quality of life and cognitive function. Therapy of choice is surgery, followed by cranial radiotherapy in about half of the patients. The standardised overall mortality rate varies 2.88-9.28 in cohort studies. Patients with CP have a 3-19 fold higher cardiovascular mortality in comparison to the general population. Women with CP have an even higher risk.

Background: We report long-term outcomes in adult patients with craniopharyngioma following surgery and radiation therapy (RT)., Material and Methods: Fifty-three patients treated with RT (median, 50 Gy in 25 fractions) between 1980 and 2009 with pathologically confirmed craniopharyngioma were reviewed (53% solid and 47% cystic/solid). The median age was 53 years (range, 22-76), 53% were female, 83% were sub-totally resected, 6% were gross totally resected and 11% had a biopsy and/or cyst aspiration alone. RT was delivered adjuvantly in 53% of patients as opposed to salvage intent upon progression., Results: Median follow-up was seven years (86 months, range, 8-259). The 5- and 10-year progression-free survival (PFS) rates were 85% and 69%, overall survival (OS) rates were 76% and 70%, and cause-specific survival (CSS) rates were both 88%, respectively. Both univariable and multivariable analysis identified age (<53 or ≥53) as a prognostic factor for OS (p =0.0003) and CSS (p =0.05). PFS was observed to be worse in patients with >2 surgeries prior to RT (p =0.01). Neither the intent of radiation or tumor type (cystic vs. solid/cystic) were prognostic or predictive. New endocrinopathies and visual dysfunction were observed in 53% and 17% of patients post-surgery, and in 11% and 6% post-RT, respectively., Conclusion: We report long-term favorable PFS, CSS and OS for craniopharyngioma post-RT. We observe age as a significant prognostic factor, however, timing of radiation was not.

Context: Craniopharyngioma (CP), Rathke's cyst (RC), and xanthogranuloma (XG) are closely related rare sellar masses. Treatment strategies in children lack consensus., Objective: Our objective was to study clinical manifestations and treatment-related outcome in RC, XG, and CP patients., Design: We conducted a multicenter surveillance trial., Patients: Inclusion criteria were 1) histological diagnosis of CP, XG, or RC and 2) diagnosis at age of 18 yr or less. A total of 117 CP, 14 XG (2001-2006), and 14 RC (1996-2006) were recruited., Main Outcome: Overall survival (OS), event-free survival (EFS), and quality of life (QoL) were evaluated., Results: The 5-yr OS rates were 1.00 ± 0.00 in RC and XG and 0.97 ± 0.02 in CP. The 5-yr EFS rates were 0.85 ± 0.10 in RC, 1.00 ± 0.00 in XG, and 0.50 ± 0.05 in CP. Surgical resection of XG results in complete remission without recurrence. Recurrences occur in RC (14%) and CR (59%) but can be efficiently treated by irradiation, reoperation, and/or intracystic treatment. Severe hypothalamic sequelae such as obesity and others affecting QoL are predominant in CP due to presurgical involvement (59%) and postsurgical lesions (44%) of posterior hypothalamic structures. Centers with lower neurosurgery patient load use more radical surgical approaches to treat CP, resulting in higher rates of obesity and reduced QoL. Despite 46% anterior hypothalamic involvement, severe obesity is not encountered in XG., Conclusions: Treatment of choice in XG and RC is radical surgery. In CP involving hypothalamic structures, less radical surgical approaches preserving hypothalamic integrity are recommended. Due to frequent relapses, regular imaging during follow-up is recommended for CP and RC. Treatment of patients with sellar masses should be confined to experienced multidisciplinary teams.

Craniopharyngioma is histologically benign and associated with high survival rates but poor quality of life. The SEER Program is among the most cited data sources regarding malignancies in the United States. SEER began collecting data regarding craniopharyngiomain 2004. SEER-STAT v7.0.5 was utilized to identify patients (January 1, 2004-December 31, 2008) with ICD-O-3 codes for craniopharyngioma. Age was categorized into 3 groups: ≤19, 20-34, and ≥35 years, as was surgical intervention: none, subtotal resection, and gross total/radical resection. Demographic, initial treatment, and follow-up data were collected. 635/662 (95.9 %) patients had complete data. Incidence per million patient-years by age group was 1.9, 1.1, and 1.9, respectively (p < 0.0001). There was bimodal incidence, with peaks at 5-9 and 60-74 years. Surgery occurred in 528 patients (83.1 %), without association between age group and extent of surgery (p = 0.14). Radiation was delivered in 139 (21.9 %) cases, with no association between treatment and extent of surgery (p = 0.73) or age group (p = 0.14). Median follow-up was 23 months. Overall and Cause-specific Survival were 87.9 and 94.5 %, respectively. Neither was associated with extent of surgery but both were positively associated with radiation (p = 0.0003 and 0.0007, respectively). There was no difference in OS or CSS when comparing STR ± RT versus GTR alone (p = 0.38 and 0.56, respectively). SEER provides reliable demographic and survival data regarding craniopharyngioma. SEER's focus on mortality statistics limits utility for outcomes studies in tumors with high survival rates, such as craniopharyngioma. Initial treatment data from SEER varies somewhat from current literature, meriting further investigation.

Clinical experience suggests that craniopharyngiomas may temporarily increase in size after radiation therapy (RT). The study goal is to determine the incidence and natural history of this response in a cohort of patients managed at Children's Healthcare of Atlanta (CHOA) or Emory Healthcare (EHC). Between 08/1998 and 06/2009, 41 children and young adults were diagnosed with craniopharyngioma at CHOA and/or EHC. Of these, 21 received external-beam radiation and were included in our analysis. Serial magnetic resonance imaging (MRI) studies were evaluated volumetrically to assess response to RT. Median age at diagnosis was 8.2 years (range 3.2-23.5 years). Median radiation dose was 54.0 Gy using standard fractionation (1.8-2.0 Gy/day). With median follow-up of 41.3 months (range 7.2-121.8 months), actuarial local control and overall survival rates at 5 years were 78.7 % and 100 %, respectively. Of subjects, 52.4 % of subjects (11 of 21) were noted on serial MRI evaluation to have tumor enlargement (mostly cystic component) after radiation before eventual shrinkage without further intervention. For tumors that expanded, the median volume increase was 33.9 % (range 15.6-224.4 %). Median time to maximal tumor/cyst expansion was 1.5 months (range 1.0-5.0 months). Finally, nearly all patients (20 of 21) showed a measurable objective response to therapy by MRI regardless of ultimate disease control. Median time to maximal response post-radiation, as defined by MRI, was 9.5 months (range 3.5-39.9 months). In summary, RT is effective for managing craniopharyngioma. However, despite good ultimate responses, approximately 50 % of the patients show tumor/cyst expansion on MRI over the first few months post-radiation. Caution should be taken not to subject these patients to "salvage surgery" or cyst aspiration during this early time unless there are other overriding surgical indications. Understanding the natural history of this phenomenon could potentially help guide the management of these craniopharyngioma patients.

Purpose: The Children's Cancer Leukaemia Group (CCLG) proposed a management pathway for craniopharyngioma that advocated limited surgery followed by upfront radiotherapy (RT) for large tumours with hypothalamic involvement and a radical resection only for smaller tumours without hypothalamic involvement. This strategy is not proven to provide optimum care or to be risk-free. The aim of this study is to review our experience of the management of craniopharyngioma diagnosed since the introduction of the CCLG guidelines in 2005., Methods: All children diagnosed with craniopharyngioma at Alder Hey Children's Hospital NHS Foundation Trust in the period between 1 January 2005 and 30 June 2011 were included. Management was based on the presence of hypothalamic syndrome, hydrocephalus, tumour size and radiological Paris grading system. Endoscopic drainage of tumour cyst was performed prior to formalising risk grade and surgical strategy. Definitive surgery was performed in 4-6 weeks time. In this respect, we developed a grading criteria., Results: Twenty patients were included. Ten of the children underwent endoscopic cyst drainage prior to definitive surgery. The results of the subsequent surgical excision were complete resection, near total resection or subtotal resection in 30, 25 and 45 % patients, respectively. There was no surgical-related mortality and no new neurological deficits. Nine patients underwent RT at some stage., Conclusions: In this study, we tried to develop an advanced model for the management of craniopharyngioma with a new risk grading system. This may have a direct impact on the surgical strategy and outcome and could be able to improve morbidity.

Craniopharyngiomas are slow growing tumors of the sellar and parasellar region and may also involve the hypothalamus. Treatment involves maximal surgical excision or subtotal resection followed by focal radiation therapy. Late effects of treatment include endocrinopathies, cognitive deficits, behavioral changes, obesity and sleep dysfunction. We conducted a retrospective review of all patients with craniopharyngioma more than 2 years off treatment and who were evaluated in the neuro-oncology survivorship clinic between 2003 and 2007. Clinical data, extent of resection, treatment modalities, endocrine status, patient symptom report and sleep study results were collected to evaluate the presence of patient reported daytime sleepiness and sleep disturbance and to determine possible risk factors. 28 patients were identified (25 %) female. 19/28 self-reported daytime fatigue or sleep disturbance; this included 4/6 patients with gross total resection and 15/22 with subtotal resection. 16/22 patients treated with cranial irradiation reported sleep-related abnormalities, compared to 3/6 patients who did not receive radiation. All but one patient had pituitary dysfunction requiring hormonal replacement. Patients with more than ≥2 sleep related complaints had a higher BMI (44.6 vs. 32.6, p = 0.0192). 8 patients underwent formal sleep evaluation. 3 patients had documented central or obstructive sleep apnea. The mean arousal index was 11.0/h (normal <5). Two patients were treated with melatonin for sleep disturbance and 2 were treated with stimulants for excessive daytime sleepiness. A majority of patients with craniopharyngioma have self-reported daytime fatigue and/or sleep dysfunction after treatment. Extent of resection did not increase the likelihood of patient-reported daytime sleepiness and/sleep dysfunction; however, patients who received radiation more frequently reported daytime sleepiness and/or sleep dysfunction. Patients with a higher BMI were more likely to experience sleep disturbance. Formal sleep evaluations should be considered in all patients with craniopharyngioma.

Recent studies suggest that subtotal resection (STR) followed by adjuvant radiation therapy is an appealing alternative to gross total resection (GTR) for craniopharyngioma, as STR provides similar tumor control without the associated endocrinological and behavioral morbidity. We have examined the impact of maximal safe resection on the clinical outcome of patients with craniopharyngioma. A total of 90 patients underwent surgical resection of craniopharyngioma at a single institution between January 1995 and April 2009. Sixty-one patients underwent GTR alone, four underwent GTR followed by adjuvant radiotherapy, 15 underwent STR alone, and 10 underwent partial removal followed by adjuvant radiotherapy. We analyzed and compared the clinical and endocrinological outcomes and radiological follow-up data of these patients. During the follow-up period, tumor recurrence following the initial resection occurred in 36 of 90 patients (40%). The repeat resection rate was higher in the STR group than the GTR group. Recurrence occurred in 20 of 61 patients (32.8%) from the GTR alone group, in 11 of 15 patients (73.3%) from the STR alone group, and in five of 10 (50%) patients from the STR with adjuvant radiation, such as radiotherapy or stereotactic radiosurgery, group (p=0.030). Maximal safe resection of craniopharyngioma leads to excellent local control. STR with adjuvant radiation therapy does not assure preservation of endocrine function, although it provides better local control than STR alone., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Background and Aims: Craniopharyngioma is a rare and mostly benign epithelial tumor of the central nervous system, mostly affecting children. Considering that most of the published series of craniopharyngioma are based on pediatric populations, studies in adults gain importance based mainly on the reduced number of cases and the possible differences emerging from a mostly different histological type. We undertook this study to establish the pattern of presentation, morphological features and specific characteristics of craniopharyngioma in an adult Mexican population, as well as discussing the long-term outcome and how it may be influenced by surgical, anatomic and clinical factors., Methods: A total of 153 adult patients (16 years or older) underwent transcranial and transsphenoidal surgery between January 1985 and December 2009, all with histological confirmation of craniopharyngioma. Hypothalamic involvement, surgical complications, rate of tumor resection and endocrinological outcome were evaluated., Results: Seventy nine males (51.6%) and 74 females (48.4%) were included. Mean age at diagnosis was 32.4 years (range: 16-77 years). Mean initial tumor volume was 28.44 mL (range: 0.18-100.44 mL). Partial or complete hypothalamic involvement (Samii Grades III, IV and V) was found in 90.2%. The overall rate of new endocrinopathies was 37.25% after surgery (95% CI = 33.9-41.2)., Conclusions: Gross total removal of craniopharyngiomas with large hypothalamic involvement was related to poor neuroendocrine outcome in adults. Partial removal should be indicated, associated with adjuvant therapy, in order to improve postoperative neuroendocrine status., (Copyright © 2012 IMSS. Published by Elsevier Inc. All rights reserved.)

The purpose of this study is to evaluate the roles of resection extent and adjuvant radiation in the treatment of craniopharyngiomas. We reviewed the records of 122 patients ages 11-52 years who received primary treatment for craniopharyngioma between 1980 and 2009 at the University of California, San Francisco (UCSF). Primary endpoints were progression free survival (PFS) and overall survival (OS). Secondary endpoints were development of panhypopituitarism, diabetes insipidus (DI), and visual field defects. Of 122 patients, 30 (24%) were treated with gross total resection (GTR) without radiation therapy (RT), 3 (3%) with GTR + RT, 41 (33.6%) with subtotal resection (STR) without RT, and 48 (39.3%) with STR + RT. Median age at diagnosis was 30 years, with 46 patients 18 years or younger. Median follow-up for all patients was 56.4 months (interquartile range 18.9-144.2 months) and 47 months (interquartile range 12.3-121.8 months) for the 60 patients without progression. Fifty six patients progressed, 10 have died, 6 without progression. Median PFS was 61.1 months for all patients. PFS rate at 2 years was 61.5% (95% CI: 52.1-70.9). OS rate at 10 years was 91.1% (95% CI 84.3-97.9). There was no significant difference in PFS and OS between patients treated with GTR vs. STR + XRT (PFS; p = 0.544, OS; p = 0.735), but STR alone resulted in significantly shortened PFS compared to STR + RT or GTR (p < 0.001 for both). STR was associated with significantly shortened OS compared to STR + RT (p = 0.050) and trended to shorter OS compared to GTR (p = 0.066). GTR was associated with significantly greater risk of developing DI (56.3 vs. 13.3% with STR + XRT, p < 0.001) and panhypopituitarism (54.8 vs. 26.7% with STR + XRT, p = 0.014). In conclusion, for patients with craniopharyngioma, STR + RT may provide superior clinical outcome, achieving better disease control than STR and limiting side effects associated with aggressive surgical resection.