Adult Craniopharyngiomas Research Articles

Adult Craniopharyngioma Treated with Radiotherapy

Adult Craniopharyngioma Treated with Radiotherapy

Outcomes after craniotomy for resection of craniopharyngiomas in adults: analysis of the National Surgical Quality Improvement Program (NSQIP).

Craniopharyngiomas occur in suprasellar locations that pose challenges for surgical management. This study evaluates the incidence of complications following craniotomy for craniopharyngioma in adults and investigates risk factors for these complications. Patients who underwent craniotomy for excision of craniopharyngioma were identified from the 2005-2016 American College of Surgeons National Surgical Quality Improvement Program (ACS-NSQIP). Incidence of 30-day postoperative complications was determined. Multivariable logistic regression identified demographic, comorbid and perioperative characteristics associated with any complication and major (Clavien IV) complications. RESULTS: There were 143 cases identified. Fifty-one (35.7%) had a complication, twenty (14.0%) experienced a major complication and there were four (2.8%) deaths. The most common complications were: unplanned readmission (13.3%), prolonged ventilation > 48h (9.8%), and unplanned reoperation (9.3%). In multivariable analysis, variables significantly associated with any complication were: black race (OR 0.16; 95% CI 0.03-0.84; p = 0.03), hypertension (OR 5.04; 95% CI 1.79-14.17; p = 0.002) and longer duration of surgery (OR 1.27; 95% CI 1.01-1.58; p = 0.04). Hypertension (OR 9.33; 95% CI 1.61-54.21; p = 0.01) and longer duration of surgery (OR 1.51; 95% CI 1.05-2.17; p = 0.03) were also significant predictors for major complications. One-third of patients undergoing craniotomy for craniopharyngioma resection experienced a postoperative complication. While high, this contrasts previously reported rates of two-thirds. Prolonged operative time and hypertension are positive predictors of major complications. This information can assist in counseling patients and decision-making for management. We note that other treatment approaches, such as endoscopic surgical techniques, radiosurgery and radiation therapy likely have different profiles and predictors of complications.

Neurological and neuropsychological outcome after resection of craniopharyngiomas.

Craniopharyngiomas are rare and benign tumors of the sellar and/or parasellar region. Primary treatment involves resection followed by adjuvant radiotherapy. While the grade of resection was frequently analyzed following surgery, the neurological outcome and especially neuropsychological deficits and quality of life have been neglected for many decades. Therefore, the authors retrospectively analyzed their patient series and prospectively assessed neuropsychological outcome and quality of life following resection of craniopharyngiomas in adults. In total, 71 patients (39 men and 32 women) with a mean age of 49 years were enrolled in the retrospective analysis. In addition, 36 of the 71 patients were included in the prospective arm of the study and underwent neurological and neuropsychological testing as well as quality of life (36-Item Short-Form Health Survey; SF-36) assessment. Factors influencing outcome were identified and correlations calculated. Resection was performed mostly using a pterional (41.6%, 47/113 surgical procedures) or bifrontal translamina terminalis (30.1%, 34/113 surgical procedures) approach. Following surgery, visual acuity was significantly improved (> 0.2 diopters) in 32.4% (23/71) of patients, or remained stable in 45.1% (32/71) of patients. During long-term follow up, 80.3% (57/71) of patients developed pituitary insufficiency, particularly involving the corticotropic and thyrotrophic axes. In total, 75% (27/36) of patients showed neuropsychological deviations in at least 1 test item. In particular, attentiveness, cognitive speed, and short-term memory were affected. Referring to the SF-36 score, quality of life was affected in both the mental and physical score in 19.4% (7/36) and 33.3% (12/36), respectively. The risk factors that were identified were a tumor volume larger than 9 cm3, tumor extension toward/into the third ventricle or the brainstem, and resection using a bifrontal translamina terminalis or left-sided approach. This study demonstrated that resection of craniopharyngiomas is frequently associated with postoperative neuropsychological deficits and hence an impaired quality of life. In addition to tumor size and extension toward/into the third ventricle or the brainstem, selection of the surgical approach may play a crucial role in the patient's neuropsychological outcome and quality of life.

Endocrine and Visual Outcomes Following Gross Total Resection and Subtotal Resection of Adult Craniopharyngioma: Systematic Review and Meta-Analysis

The optimal surgical strategy for management of adult patients with craniopharyngioma remains controversial. To analyze the functional outcomes ofadult patients with gross total resection (GTR) and subtotal resection (STR) of craniopharyngioma. MEDLINE, EMBASE, Scopus, and Cochrane databases were searched from inception to July 19, 2018, for articles comparing postoperative endocrine function, vision, complications, and recurrence rates for adult patients with GTR and STR of craniopharyngioma. The articles were analyzed by meta-analysis of proportions using a random-effects model to calculate summary odds ratios (ORs). The initial search resulted in 2468 studies and 540 studies selected for full text review. Seventeen studies were included in the final analyses with 748 patients in the GTR cohort and 559 patients in the STR cohort. GTR resulted in a significantly lower likelihood of recurrence when compared with STR (OR, 0.106; 95% confidence interval [CI], 0.067-0.168; P < 0.001), but a significantly greater likelihood of panhypopituitarism (OR, 2.063; 95% CI, 1.058-4.024; P= 0.034) and permanent diabetes insipidus (OR, 2.776; 95% CI, 1.321-5.832; P= 0.007). There was no significant difference between the groups for postoperative worsened vision (P= 0.868), improved vision (P= 0.876), pathologic weight gain (P= 0.724), cerebrospinal fluid leak (P= 0.788), complications (P= 0.656), or death (P= 0.261). This is the first systematic review of functional outcomes of adult patients with craniopharyngioma. GTR results in decreased likelihood of recurrence, but increased likelihood of postoperative panhypopituitarsm and permanent diabetes insipidus. Surgeons should be aware of these associations when determining the optimal operative strategy for adult patients with craniopharyngioma.

Adult Craniopharyngioma: Case Series, Systematic Review, and Meta-Analysis.

The optimal therapeutic approach for adult craniopharyngioma remains controversial. Some advocate for gross total resection (GTR), while others advocate for subtotal resection followed by adjuvant radiotherapy (STR + XRT). To conduct a systematic review and meta-analysis assessing the rate of recurrence in the follow-up of 3 yr in adult craniopharyngioma stratified by extent of resection and presence of adjuvant radiotherapy. MEDLINE (1946-July 1, 2016) and EMBASE (1980-June 30, 2016) were systematically reviewed. From1975 to 2013, 33 patients were treated with initial surgical resection for adult onset craniopharyngioma at our center and were reviewed for inclusion in this study. Data from 22 patients were available for inclusion as a case series in the systematic review. Eligible studies (n = 21) were identified from the literature in addition to a case series of our institutional experience. Three groups were available for analysis: GTR, STR + XRT, and STR. The rates of recurrence were 17%, 27%, and 45%, respectively. The risk of developing recurrence was significant for GTR vs STR (odds ratio [OR]: 0.24, 95% confidence interval [CI]: 0.15-0.38) and STR + XRT vs STR (OR: 0.20, 95% CI: 0.10-0.41). Risk of recurrence after GTR vs STR + XRT did not reach significance (OR: 0.63, 95% CI: 0.33-1.24, P = .18). This is the first and largest systematic review focusing on the rate of recurrence in adult craniopharyngioma. Although the rates of recurrence are favoring GTR, difference in risk of recurrence did not reach significance. This study provides guidance to clinicians and directions for future research with the need to stratify outcomes per treatment modalities.

Benefits of image-guided stereotactic hypofractionated radiation therapy as adjuvant treatment of craniopharyngiomas. A review.

Craniopharyngiomas account for 5.6-13% of intracranial tumors in children. Despite being histologically benign, these tumors remain a major neurosurgical challenge because of the typical tight adherence to adjacent critical structures. The optimal therapeutic approach for this disease is controversial. Large cystic size and adherence to neurovascular, neuroendocrine, and optic structures without a clear line of cleavage make complete resection problematic and often hazardous. For these reasons, partial resection and adjuvant treatment play an important role. Post-operative radiation therapy (RT) following either complete or incomplete tumor removal is associated with significantly decreased recurrence rates. The aim of this review is to analyze the potential advantage of the most modern technical advancements for RT of craniopharyngiomas. This narrative review on the topic of craniopharyngiomas was based on published data available on PUBMED/Medline. All data concerning adjuvant or upfront radiation therapy treatment of craniopharyngioma were reviewed and summarized. A more detailed analysis of fractionated frameless steretactic radiosurgery of these tumors is provided as well. We reviewed the possible improvement provided by intensity modulated beams, arc therapy, image guidance, proton radiation, and fractionated stereotactic radiosurgery. Many published findings on outcome and toxicity after RT involve the use of relatively outdated RT techniques. Technologic improvements in imaging, radiation planning, and delivery have improved the distribution of radiation doses to desired target volumes and reduced the dose to nearby critical normal tissues. Currently available techniques, providing image guidance and improved radiation doses distribution profile, have shown to maintain the efficacy of conventional techniques while significantly reducing the toxicity. Image-guided radiosurgery holds the dose distributions and precision of frame-based techniques with the remarkable advantage of multiple-session treatments that are better tolerated by sensitive peritumoral structures, such as the optic pathway and hypothalamus. This, together with the comfort of a frameless technique, candidates frameless image-guided radiosurgery to be the first option for the adjuvant post-operative treatment of craniopharyngiomas in children and young adults when total resection cannot be achieved, in particular those with hypothalamic involvement, and when the residual tumor is mostly solid.

The limits of transsellar/transtuberculum surgery for craniopharyngioma.

The proximity of craniopharyngiomas to vital neurovascular structures and their high recurrence rates make them one of the most challenging brain tumors to treat. Although surgery remains the first line of therapy and offers the best chance of radical resection and oncological cure, the high recurrence tendency of craniopharyngiomas, even after apparent total removal, often makes adjuvant treatment essential. The endoscopic endonasal approach (EEA) has been recently introduced as a treatment option for both pediatric and adult craniopharyngiomas, rapidly gaining wide acceptance over the traditional transcranial approaches. Although the primary role of EEA over traditional transcranial approaches has been slowly accepted in the literature, little has been written about the limitations and potential contraindications of this approach in the treatment of craniopharyngiomas. This article presents the advantages and highlights the limitations of endoscopic transsellar/transtuberculum surgery for craniopharyngiomas. In every case, surgery should be tailored to individuals based on their age and comorbidities, presenting symptoms, tumor characteristics, prior treatment and treatment tolerance, as well as the surgeon's preference based on personal experience and comfort.

Adult Craniopharyngioma: Case Series, Systematic Review, and Meta-analysis

Background The optimal therapeutic approach for adult craniopharyngioma remains controversial. Some advocate for gross total resection (GTR), while others advocate for subtotal resection followed by adjuvant radiotherapy (STR + XRT). Objective To conduct a systematic review and meta-analysis assessing the rate of recurrence in the follow-up of 3 yr in adult craniopharyngioma stratified by extent of resection and presence of adjuvant radiotherapy. Methods MEDLINE (1946-July 1, 2016) and EMBASE (1980-June 30, 2016) were systematically reviewed. From1975 to 2013, 33 patients were treated with initial surgical resection for adult onset craniopharyngioma at our center and were reviewed for inclusion in this study. Results Data from 22 patients were available for inclusion as a case series in the systematic review. Eligible studies (n = 21) were identified from the literature in addition to a case series of our institutional experience. Three groups were available for analysis: GTR, STR + XRT, and STR. The rates of recurrence were 17%, 27%, and 45%, respectively. The risk of developing recurrence was significant for GTR vs STR (odds ratio [OR]: 0.24, 95% confidence interval [CI]: 0.15-0.38) and STR + XRT vs STR (OR: 0.20, 95% CI: 0.10-0.41). Risk of recurrence after GTR vs STR + XRT did not reach significance (OR: 0.63, 95% CI: 0.33-1.24, P = .18). Conclusion This is the first and largest systematic review focusing on the rate of recurrence in adult craniopharyngioma. Although the rates of recurrence are favoring GTR, difference in risk of recurrence did not reach significance. This study provides guidance to clinicians and directions for future research with the need to stratify outcomes per treatment modalities.

Endoscopic endonasal approach for craniopharyngioma: the importance of the relationship between pituitary stalk and tumor.

OBJECTIVE The endoscopic endonasal approach (EEA) is commonly used for the treatment of craniopharyngioma; therefore, it is essential to analyze outcomes in order to understand the benefits and drawbacks. The goal of this paper was to evaluate the clinical features and outcomes associated with this treatment approach. METHODS From July 2010 to March 2016, 82 adult craniopharyngioma patients underwent an EEA at the authors' institution. Of these cases, intraoperative records and immediate postoperative MR images were available for 68 patients. The patients underwent systemized endocrinological evaluation. Eighteen of 68 patients who underwent EEA for recurrence or regrowth of residual lesions after previous surgical management were excluded in the analysis of the anatomical tumor classification. The authors retrospectively analyzed preoperative clinical features and previous anatomical classifications, focusing on the relationship of the pituitary stalk and tumor, to determine predictive factors for the clinical outcome, such as the extent of resection, visual function, endocrinological function, recurrence rate, and complications. RESULTS The mean tumor size was 2.5 cm (3.1 cm for primary tumors and 1.9 cm for recurrent lesions). Gross-total resection (GTR) was achieved in 62 (91.1%) patients (48 [96.0%] patients with primary tumors and 14 [77.8%] patients with recurrent tumors). The rate of GTR was higher in the primary group than in the group with recurrence (p = 0.038). The overall pre- and postoperative visual impairment scale (VIS) scores were 40.8 and 22.1, respectively (50.9 and 14.3 in the primary group and 30.7 and 29.9 in patients with recurrence, respectively). The improvement rate in VIS score was higher in the primary group than in the recurrent group (p = 0.001). Endocrinological function was improved in 4 patients (5.9%) and deteriorated in 32 of 68 patients (47.1%). Tumor invasion into the center of the pituitary stalk affected the postoperative outcomes most significantly. Cognitive dysfunction was observed in 22 patients before surgery and improved in 20 patients (90.9%) after surgery. Hydrocephalus was found in 7 patients and resolved after surgery in all cases. CSF leakage occurred in 2 (2.9%) of 68 patients and was repaired by revision surgery in both patients. Ten patients without CSF leakage also received antibiotics for the treatment of meningitis. The infection rate was higher in the recurrent group. Postoperative endocrinological evaluation showed no deficits in 12 patients and panhypopituitarism in 55 patients. The remaining patient had growth hormone deficiency. Forty-three patients had new-onset diabetes insipidus, and 1 patient had persistent diabetes insipidus after surgery. There were 2 (2.9%) cases of recurrence during the mean 30.7-month follow-up period; one patient underwent radiosurgery and the other underwent reoperation. CONCLUSIONS The EEA resulted in excellent surgical outcomes and acceptable morbidity rates, regardless of the anatomical location of the tumor. Invasion of the craniopharyngioma into the center of the pituitary stalk has strong predictive power for postoperative endocrinological outcome.

P.041 Adult craniopharyngioma: case series, systematic review and meta-analysis

Background: The optimal therapeutic approach for adult craniopharyngioma remains controversial. Some advocate for gross total resection (GTR), while others support subtotal resection followed by adjuvant radiotherapy (STR + XRT). Methods: MEDLINE (1946 to July 1st 2016) and EMBASE (1980 to June 30th 2016) were systematically reviewed. Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guideline was followed. At our institution, from 1975 to 2013, 33 patients were treated with initial surgical resection for adult onset craniopharyngioma. 22 patients were included in the present case series. Results: Eligible studies (n=21) were identified from the literature in addition to a case series of our institutional experience. Three groups were available for analysis: GTR, STR + XRT, and STR. The rates of recurrence were 17 %, 27 % and 45%, respectively. This differs from childhood population. The difference in risk of recurrence after GTR vs. STR + XRT did not reach significance (OR: 0.63, 95% confidence interval: 0.33-1.24, P=0.18). This maybe because of low pooled sample size (n=99) who underwent STR+XRT. Conclusions: This is the first and largest meta-analysis examining rate of recurrence in adult craniopharyngioma. Thus, when safe and feasible, a goal of gross total resection should be favored. Each patient should be considered on a case-by-case basis.

EP-1120: Fractionated stereotactic radiotherapy in adult craniopharyngiomas : outcomes and complications

EP-1120: Fractionated stereotactic radiotherapy in adult craniopharyngiomas : outcomes and complications

Visual outcome, endocrine function and tumor control after fractionated stereotactic radiation therapy of craniopharyngiomas in adults: findings in a prospective cohort

Background: The purpose of this study was to examine visual outcome, endocrine function and tumor control in a prospective cohort of craniopharyngioma patients, treated with fractionated stereotactic radiation therapy (FSRT).Material and methods: Sixteen adult patients with craniopharyngiomas were eligible for analysis. They were treated with linear accelerator-based FSRT during 1999–2015. In all cases, diagnosis was confirmed by histological analysis. The prescription dose to the tumor was 54 Gy (median, range 48–54) in 1.8 or 2.0 Gy per fraction, and the maximum radiation dose to the optic nerves and chiasm was 54.2 Gy (median, range 48.6–60.0) for the cohort. Serial ophthalmological and endocrine evaluations and magnetic resonance imaging (MRI) scans were performed at regular intervals. Median follow-up was 3.3 years (range 1.1–14.1), 3.7 years (range 0.8–15.2), and 3.6 years (range 0.7–13.1) for visual outcome, endocrine function, and tumor control, respectively.Results: Visual acuity impairment was present in 10 patients (62.5%) and visual field defects were present in 12 patients (75%) before FSRT. One patient developed radiation-induced optic neuropathy at seven years after FSRT. Thirteen of 16 patients (81.3%) had pituitary deficiency before FSRT, and did not develop further pituitary deficiency after FSRT. Mean tumor volume pre-FSRT was 2.72 cm3 (range 0.20–9.90) and post-FSRT 1.2 cm3 (range 0.00–13.10). Tumor control rate was 81.3% at two, five, and 10 years after FSRT.Conclusions: FSRT was relatively safe in this prospective cohort of craniopharyngiomas, with only one case of radiation-induced optic neuropathy and no case of new endocrinopathy. Tumor control rate was acceptable.

Patterns of care and treatment outcomes of patients with Craniopharyngioma in the national cancer database.

To investigate the patterns of care and outcomes in patients with craniopharyngioma in the National Cancer Data Base (NCDB). This study included 697 patients (166 pediatric and 531 adult cases) treated for craniopharyngioma between 2004 and 2012 in the NCDB. Adjuvant radiotherapy (RT) was defined if within 6 months of surgery. Limited surgery (LS) was defined as biopsy or subtotal resection. Proportional-hazards models were used to evaluate associations between covariates and overall survival (OS). A time-dependent analysis of RT was performed to account for early deaths after surgery. Median follow-up was 46 months. Overall, 21% of patients received adjuvant RT. Of patients with known surgical extent (n = 195), 71% had LS. Utilization of adjuvant RT increased from 18% in 2004-2007 to 24% in 2008-2012. Patterns of care regarding adjuvant RT or LS were not significantly different between adult and pediatric patients. Tumor size, low comorbidity, and LS were associated with increased utilization of adjuvant RT. The 5-year OS among patients treated with LS, LS+RT, and gross total resection were 75, 85, and 82% (p = 0.02). On multivariate analysis of the 195 patients with known surgical extent, LS+RT was associated with improved OS compared to LS (HR 0.22, 95% CI 0.05-0.99, p = 0.04), but was not significant when early deaths (<2months from surgery) were removed to adjust for immortal-time bias. Medical practice regarding surgical approach and adjuvant RT are similar for pediatric and adult craniopharyngiomas. Immortal-time bias may confound assessment of OS for adjuvant RT. Prospective studies comparing adjuvant RT versus observation after LS are warranted.

Management and outcome of recurrent adult craniopharyngiomas: an analysis of 42 cases with long-term follow-up.

OBJECTIVE The treatment of recurrent and residual craniopharyngiomas is challenging. In this study the authors describe their experience with these tumors and make recommendations on their management. METHODS The authors performed an observational study of adult patients (≥ 18 years) with recurrent or residual craniopharyngiomas that were managed at their tertiary center. Retrospective data were collected on demographics and clinical, imaging, and treatment characteristics from patients who had a minimum 2-year follow-up. Descriptive statistics were used and the data were analyzed. RESULTS There were 42 patients (27 male, 15 female) with a mean age of 46.3 ± 14.3 years. The average tumor size was 3.1 ± 1.1 cm. The average time to first recurrence was 3.6 ± 5.5 years (range 0.2-27 years). One in 5 patients (8/42) with residual/recurrent tumors did not require any active treatment. Of the 34 patients who underwent repeat treatment, 12 (35.3%) had surgery only (transcranial, endoscopic, or both), 9 (26.5%) underwent surgery followed by adjuvant radiation therapy (RT), and 13 (38.2%) received RT alone. Eighty-six percent (18/21) had a gross-total (n = 4) or near-total (n = 14) resection of the recurrent/residual tumors and had good local control at last follow-up. One of 5 patients (7/34) who underwent repeat treatment had further treatment for a second recurrence. The total duration of follow-up was 8.6 ± 7.1 years. The average Karnofsky Performance Scale score at last follow-up was 80 (range 40-90). There was 1 death. CONCLUSIONS Based on this experience and in the absence of guidelines, the authors recommend an individualized approach for the treatment of symptomatic or growing tumors. This study has shown that 1 in 5 patients does not require repeat treatment of their recurrent/residual disease and can be managed with a "scan and watch" approach. On the other hand, 1 in 5 patients who had repeat treatment for their recurrence in the form of surgery and/or radiation will require further additional treatment. More studies are needed to best characterize these patients and predict the natural history of this disease and response to treatment.

Role of Definitive Radiation Therapy in Adult Craniopharyngioma Patients

Role of Definitive Radiation Therapy in Adult Craniopharyngioma Patients

Evaluation of Macular Ganglion Cell Complex and Peripapillary Retinal Nerve Fiber Layer in Primary Craniopharyngioma by Fourier-Domain Optical Coherence Tomography

BackgroundThe aim of this study was to compare the differences in macular ganglion cell complex (GCC) and peripapillary retinal nerve fiber layer (pRNFL) in child and adult patients with primary craniopharyngioma by Fourier-domain optical coherence tomography (FD-OCT) and to evaluate their significance in the diagnosis of primary craniopharyngioma.Material/MethodsNinety-six participants were divided into 3 groups: 32 in the child craniopharyngioma group (CCG) and 32 in the adult craniopharyngioma group (ACG) who were treated in Beijing Tiantan Hospital between November 2013 and October 2014, and 32 in the normal group (NG). All subjects were scanned by FD-OCT to map GCC and pRNFL thicknesses. Spearman correlation coefficient was used to assess the correlation between GCC and pRNFL thickness, and pRNFL thickness and optic nerve head (ONH) parameters, including horizontal cup-disc ratio (HCDR), vertical cup-disc ratio (VCDR), optic disc area (ODA), and cup area (CA), respectively.ResultsThe correlation between GCC and pRNFL thickness in the CCG was slightly stronger compared with the ACG. A significant difference in GCC thickness was observed among the CCG, ACG, and NG. Although the pRNFL thickness in both the CCG and ACG was significantly higher than that in NG, no significant difference in pRNFL thickness was detected between the 2 craniopharyngioma groups. The average, superior, and inferior pRNFL thicknesses were negatively correlated with VCDR in the CCG (in double eyes) and ACG (only in left eyes).ConclusionsGCC was more sensitive than pRNFL in detecting optic nerve damage in the eyes of craniopharyngioma patients. A thinner pRNFL was especially correlated with VCDR in child craniopharyngioma patients.

Long-term quality of life after endonasal endoscopic resection of adult craniopharyngiomas.

Craniopharyngiomas are benign parasellar tumors for which surgical removal, although potentially curative, often leads to morbidity with resulting decreases in quality of life (QOL). The endonasal endoscopic approach is a minimal-access technique for removing these tumors and may reduce postoperative morbidity. The QOL following this method for resection of craniopharyngiomas has not been documented. The authors reviewed a database of consecutive endonasal endoscopic surgeries done at Weill Cornell Medical College. Adult patients with histologically proven craniopharyngiomas were included who had completed either only postoperative (> 9 months) or both pre- and postoperative QOL forms, the Anterior Skull Base Quality of Life (ASBQ) questionnaire, and the 22-Item Sinonasal Outcome Test (SNOT-22). Rates of gross-total resection (GTR), complications, and visual and endocrine function were collected. Retrospective independence (Wen score) was also assigned. A contemporaneous group of patients undergoing endonasal endoscopic pituitary macroadenoma resection was used as a control. This study included 33 procedures performed in 31 patients. The average postoperative ASBQ score was 3.35 and the SNOT-22 score was 19.6. Better QOL was associated with GTR and postoperative radiation. Worse QOL was associated with persistent visual defects, hypopituitarism, tumor recurrence, increase in body mass index, and worsening Wen score. In a subset of 10 patients, both pre- and postoperative (> 9 months) QOL scores were obtained. Both ASBQ and SNOT-22 scores showed stability and a trend toward improvement, from 2.93 ± 0.51 to 2.96 ± 0.47 (ASBQ) and 23.7 ± 10.8 to 18.4 ± 11.6 (SNOT-22). Compared with 62 patients undergoing endoscopic pituitary macroadenoma resection, patients with craniopharyngiomas had worse postoperative QOL on the ASBQ (3.35 vs 3.80; p = 0.023) and SNOT-22 (19.6 vs 13.4; p = 0.12). This report of validated site-specific QOL following endoscopic surgery for craniopharyngiomas shows an overall maintenance of postoperative compared with preoperative QOL. Better QOL could be seen in patients with GTR and radiation therapy, and worse QOL was found in patients with visual or endocrine deficits. Nevertheless, patients with craniopharyngiomas still had worse QOL than those undergoing similar surgery for pituitary macroadenomas, confirming the worse prognosis of craniopharyngiomas even when removed via a minimally invasive approach. These measures should serve as benchmarks for comparison with open transcranial approaches to similar tumors.

Excess mortality and morbidity in patients with craniopharyngioma, especially in patients with childhood onset: a population-based study in Sweden.

Craniopharyngiomas (CPs) in adults have been associated with excess mortality. The aim of the study was to investigate mortality and morbidity in patients with childhood-onset and adult-onset CP. 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. This was a nationwide population-based study. 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%. There were no interventions. Standardized mortality ratios (SMRs) and standardized incidence ratios (SIRs) with 95% confidence intervals were calculated using the Swedish population as the reference. 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). 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.

National treatment trends, complications, and predictors of in-hospital charges for the surgical management of craniopharyngiomas in adults from 2007 to 2011.

Treatment of craniopharyngiomas is one of the most demanding and controversial neurosurgical procedures performed. The authors sought to determine the factors associated with hospital charges and fees for craniopharyngioma treatment to identify possible opportunities for improving the health care economics of inpatient care. The authors analyzed the hospital discharge database of the Nationwide Inpatient Sample (NIS) covering the period from 2007 through 2011 to examine national treatment trends for adults (that is, those older than 18 years) who had undergone surgery for craniopharyngioma. To predict the drivers of in-hospital charges, a multistep regression model was developed that accounted for patient demographics, acuity measures, comorbidities, hospital characteristics, and complications. The analysis included 606 patients who underwent resection of craniopharyngioma; 353 resections involved a transsphenoidal approach (58%) and 253 a transfrontal approach (42%). The mean age (± SD) of patients was 47.7 ± 16.3 years. The average hospital length of stay (LOS) was 7.6 ± 9 days. The mean hospital charge (± SD) was $92,300 ± $83,356. In total, 48% of the patients experienced postoperative diabetes insipidus or an electrolyte abnormality. A multivariate regression model demonstrated that LOS, hospital volume for the selected procedure, the surgical approach, postoperative complications, comorbidities, and year of surgery were all significant predictors of in-hospital charges. The statistical model accounted for 54% of the variance in in-hospital charge. This analysis of inpatient hospital charges in patients undergoing craniopharyngioma surgery identified key drivers of charges in the perioperative period. Prospective studies designed to evaluate the long-term resource utilization in this complex patient population would be a useful future direction.

Recurrent craniopharyngiomas in children and adults: long-term recurrence rate and management

The significance of the majority of the factors influencing the recurrence rate (RR) of craniopharyngiomas remains unclear, and the management of this significance is controversial. The present study aimed to evaluate the influence of patient age and tumor topography on the RR, the efficacy of radiotherapy, and the safety of surgery for recurrences. The RR was analyzed in 38 children (follow-up, 2-256 months [mean, 147.6]) and 63 adults (follow-up, 2-221 months [mean, 100.2]. The efficacy of 18 sessions of radiotherapy (13 patients) and the outcome of 52 secondary surgeries (37 patients) were evaluated. The RR reached 39.5% in children and 22.2% in adults (p = 0.053). After radical tumor removal, the RR in children (36.7%) was significantly higher (p = 0.024) than that in adults (14%). In children after radical removal of intraventricular and extraventricular craniopharyngiomas (IECs), the RR was higher (60%; p = 0.071) than in extraventricular (intrasellar and suprasellar; purely suprasellar extraventricular) tumors (25%). Radical removal of 50% of tumors was achieved (73.1% in children; 26.9% in adults; p = 0.002) in 56.7% of the first and 40.9% of further recurrences. There was no early mortality after 52 surgeries; functional worsening (endocrine, 2; obesity, 2; visual, 3) occurred after 7/52 secondary surgeries. Recurrence occurred after 9/18 sessions of radiotherapy. The RR was higher in children than in adults and in IECs relative to other topographic groups. Children with IECs represent a risk group. The efficacy of radiotherapy was inconclusive. Early detection of recurrences enabled safe excision with low morbidity.