Survivors Of Craniopharyngioma Research Articles

THU220 Legal Degree Of Disability In Childhood-Craniopharyngioma Survivors After Follow-up - Results Of The Hit-Endo Study

Abstract Disclosure: S. Boekhoff: None. M. Eveslage: None. J. Beckhaus: None. C. Friedrich: None. H.L. Muller: None. Craniopharyngiomas are rare low-grade embryonic malformational tumors of the sellar/parasellar area. After a diagnosis during childhood and adolescence, endocrine and hypothalamic long-term sequalae affect the prognosis. Financial resources for psychological rehabilitation and involvement of craniopharyngioma survivors are made available by a legal status of the degree of disability (GdB), according to the German Social Code Book V. This study analyzed the relationship between clinical/psychosocial traits and quality of life (QoL) measures and the resulting GdB. The HIT-Endo study included pediatric craniopharyngioma patients (%lt;18 years of age at the time of diagnosis) and diagnosed before the year 2000. The GdB was evaluated in a cohort of 108 patients following a median follow-up time of 16 years. The GdB at follow-up was evaluated in relation to endocrine, ophthalmological, neuropsychological, and psychosocial factors. 44 patients (41%) did not receive a GdB, three patients (3%) received a GdB of 30-40, 43 patients (40%) a GdB of 50-90 and 18 patients (17%) the maximal GdB of 100. Higher GdB was associated with lower levels education, higher body mass index (BMI SDS), more severe visual impairment, and hypothalamic involvement of the tumor. Patients with a GdB of 100 reported impairments in social and occupational settings, as well as loss of physical and cognitive function, fatigue, dyspnea, and pain problems. In addition, they performed worse on the German Daily Life Ability Scale (FMH) than individuals with lower GdBs. The GdB reflects the long-term effects of craniopharyngioma during childhood and adolescence and is linked to physical and psychosocial problems. A low functional capacity, as measured by FMH scale, may indicate the eligibility for a higher GdB of craniopharyngioma survivors. Presentation: Thursday, June 15, 2023

Caregiver Burden and Its Relationship to Health-Related Quality of Life in Craniopharyngioma Survivors.

Craniopharyngioma (CP) is a rare brain tumor associated with severe comorbidities that reduce survivor health-related quality of life (HRQOL). However, CP impact on caregivers is unknown. To measure caregiver burden and examine its relationship to survivor HRQOL and other determinants in CP. Eighty-two participants who self-identified as caregivers of CP survivors responded to an online survey including caregiver-reported Pediatric Quality of Life Inventory (PedsQL), and the Zarit Burden Interview (ZBI). Caregivers reported an average of 13 out of 29 health conditions in survivors following tumor treatment, including excess weight, hypopituitarism, fatigue, mood, cognition, social issues, temperature dysregulation, visual impairment, and sleep problems. Strikingly, 70% of survivors who experienced obesity also experienced hyperphagia. ZBI scores were moderate with a median of 37. PedsQL total scores were poor with a median of 46.2. ZBI scores were independent of caregiver level of education and care duration. Both scores were independent of income, survivor age, gender, age at diagnosis, or tumor recurrence. In contrast, both scores depended on the number (P < .001) and the type of survivor health problems, with significantly worse scores for caregivers or survivors with symptoms of hypothalamic dysfunction (P < .001) including hyperphagia but not obesity. PedsQL total scores significantly predicted ZBI scores (P < .001). Survivor poly-symptomatology predicted and incurred significant caregiver burden. Our study separated hyperphagia and obesity and identified hyperphagia and other hypothalamic dysfunction symptoms as understudied issues. Altogether, these findings draw particular attention to the unmet needs of CP survivors and their caregivers.

Comparison of neurocognitive and quality-of-life outcomes in pediatric craniopharyngioma patients treated with partial resection and radiotherapy versus gross-total resection only.

The optimal management of pediatric craniopharyngioma patients remains controversial, shifting from radical resection (gross-total resection [GTR]) to a more conservative approach with partial resection/biopsy followed by radiotherapy (PR+RT). To the authors' knowledge, no previous studies have compared neurocognitive and quality-of-life (QOL) outcomes between the two main treatments. In this study, the authors compared changes in intellectual, adaptive, and QOL scores in children treated for craniopharyngioma with GTR and those treated with PR+RT. Patients underwent annual neurocognitive and QOL evaluations for up to 10 years posttreatment, including the Full-Scale IQ, Verbal Comprehension Index (VCI), Perceptual Reasoning Index (PRI), Working Memory Index (WMI), and Processing Speed Index (PSI). Child- and parent-reported QOL scores and adaptive behavior in different domains were assessed. General linear mixed models were used to examine change in scores over time by treatment group with adjustment for significant covariates. Scores from 43 patients treated between 2009 and 2019 (21 GTR, 22 PR+RT) were examined. Within the PR+RT group, 9 patients had intensity-modulated RT and 13 had proton beam therapy. The treatment groups were similar in sex (44% male) and age (median 7.3 years). There were no significant differences in the trajectory of intellectual functioning or QOL scale scores between the two groups. However, patients who underwent GTR exhibited significant improvement over time in overall adaptive behavior (p = 0.04) and conceptual skills (p = 0.01), which was not observed in patients treated with PR+RT. Long-term pediatric craniopharyngioma survivors treated with GTR and PR+RT have similar intellectual function and QOL. Larger studies are needed to explore small but clinically significant differences between the two groups.

Legal Degree of Disability in Childhood-Craniopharyngioma Survivors During Long-Term Follow-Up

Cranioparyngiomas are rare low-grade embryonic malformational tumors of the sellar/parasellar region. The prognosis after diagnosis during childood and adolescence is influenced by (neuro)endocrine long-term sequelae. A legal status of the degree of disability (GdB), according to the German Social Code Book V that is worthy of support provides financial means for psychosocial integration and participation of craniopharyngioma survivors. HIT-Endo is a German registry study on craniopharyngioma patients aged≤18 years at diagnosis . In a sample of 108 patients, the degree of disability and the association with endocrine, ophthalmological, neuropsychological (QLQ-C30; MFI-20; FMH-scale) and psychosocial parameters was analyzed after a mean follow-up period of 16 years. 47 patients (43%) did not receive a GdB or received a GdB of 30-40, 43 patients (40%) a GdB of 50-90 and 18 patients (17%) the maximal GdB of 100. Higher GdB were associated with lower education, higher body mass index standard deviation and a higher degree of visual impairment and hypothalamic involvement of the craniopharyngioma. Patients with a GdB of 100 reported loss in physical and cognitive function, dyspnea, and pain (QLQ-C30), as well as fatigue (MFI-20), and limitations in social and occupational contexts. They further had a lower functional capacity (German daily life ability scale (FMH)) compared to those with a smaller GdB. The GdB is associated with psychosocial and physical impairments and reflects the long-term consequences of craniopharyngioma. A low functional capacity may indicate a high GdB in later life of craniopharyngioma survivors.

RARE-05. Legal degree of disability in childhood-craniopharyngioma survivors during long-term follow-up – results of the HIT-ENDO study

BACKGROUND: Cranioparyngiomas are rare low-grade embryonic malformational tumors of the sellar/parasellar region. The prognosis after diagnosis during childhood and adolescence is influenced by endocrine and hypothalamic long-term sequelae. A legal status of the degree of disability (GdB), according to the German Social Code Book V that is worthy of support, provides financial means for psychosocial rehabilitation and participation of craniopharyngioma survivors. The aim of this study was to determine the association of clinical/psychosocial characteristics and quality of life (QoL) indicators with the resulting GdB. PATIENTS AND METHODS: HIT-Endo is a German registry study on craniopharyngioma patients aged ≤ 18 years at diagnosis and included before the year 2000. In a sample of 108 patients, the degree of disability and the association with endocrine, ophthalmological, neuropsychological and psychosocial parameters was analyzed after a mean follow-up period of 16 years (95% CI: 9.8-36.4). RESULTS: 44 patients (41%) did not receive a GdB, three patients (3%) received a GdB of 30-40, 43 patients (40%) a GdB of 50-90 and 18 patients (17%) the maximum GdB of 100. Higher GdB were associated with lower education, higher body mass index standard deviation and a higher degree of visual impairment and hypothalamic involvement of the craniopharyngeoma. Patients with a GdB of 100 reported loss in physical and cognitive function, as well as fatigue, dyspnea, and pain conditions, and limitations in social and occupational contexts. They further had a lower functional capacity (German daily life ability scale (FMH)) compared to those with a smaller GdB. CONCLUSION: The GdB is associated with psychosocial and physical impairments and reflects the long-term consequences of craniopharyngioma during childhood and adolescence. A low functional capacity (assessed by FMH) may indicate the eligibility for a high GdB in later life of craniopharyngioma survivors.

RARE-13. Clinical management and functional and survival outcomes in pediatric craniopharyngioma, a patient and family perspective

Craniopharyngiomas are rare, histologically benign, sellar/parasellar tumors with significant tumor and therapy related morbidity and impairment in quality of life (QOL). We report survey results from patients/families affected by childhood-onset craniopharyngioma to identify opportunities for improvement in management. An anonymous REDCap survey was distributed via social media and clinic visits to patients/families of craniopharyngioma survivors. Survey questions investigated perspectives on clinical management and functional and survival outcomes at initial diagnosis and recurrence. A total of 159 patients/families completed the survey, 40% (n=64) reported craniopharyngioma recurrence. For primary craniopharyngioma, maximal safe resection was the most frequent treatment reported (n=84), followed by partial resection (n=40), radiation (n=8), biopsy (n=5), and chemotherapy (n=3). Most patients (n=120) decided on a treatment plan within one week, 63 (40%) decided in one day. For recurrent craniopharyngioma, maximal safe resection and radiation were the most frequent interventions (n=33 each), followed by partial resection (n=13), chemotherapy (n=4) and biopsy (n=2). Multiple treatment options and/or participation in a clinical trial were offered to similar numbers of patients across primary and recurrent diagnoses (~21% for each). Most recurrent craniopharyngioma patients decided on management within one week (n=43). Long term effects related to tumor and treatment were identified as the primary concern in all respondents. The most common deficits for all patients were neuro-endocrine followed by vision and neurocognition problems. Neuro-endocrine complications were self-reported as the biggest impact on QOL. Families reported that they would prefer treatment options with the potential for improved QOL, even if these options also carried an increased risk of recurrence. Craniopharyngioma continues to be predominantly treated with surgery and radiation initially and with recurrence. Survivors have multiple comorbidities, with an interest in targeted therapies that preserve QOL. Novel therapies to prevent co-morbidities and provide long term benefits are necessary and upcoming.

Cardiac remodeling in patients with childhood-onset craniopharyngioma\u2014results of HIT-Endo and KRANIOPHARYNGEOM 2000/2007

Hypothalamic obesity caused by childhood-onset craniopharyngioma results in long-term cardiovascular morbidity. Knowledge about clinical markers and risk factors for cardiovascular morbidity is scarce. A cross-sectional study on transthoracic echocardiographic parameters was performed to determine the associations with clinical and anthropometric parameters in 36 craniopharyngioma patients. BMI correlated with the thickness of interventricular septum in diastole (IVSd) (r = 0.604, p < 0.001) and left ventricular posterior wall thickness in diastole (LVPWd) (r = 0.460, p = 0.011). In multivariate analyses on risk factors for cardiac remodeling, sex hormone replacement therapy, BMI, and male gender were positively correlated with increased left ventricular internal diameter in diastole (LVIDd), R2 = 0.596, F = 10.323, p < 0.001. BMI and insulin resistance were selected as significant independent determinants of IVSd, produced R2 = 0.655, F = 29.441, p < 0.001. Due to a wide range of disease duration, 17 pediatric and 19 adult patients were analyzed separately. In the adult subgroup (age at study ≥ 18 years), BMI correlated with IVSd (r = 0.707, p = 0.003), LVPWd (r = 0.592, p = 0.020), and LVIDd (r = 0.571, p = 0.026). In the pediatric subgroup (age at study < 18 years), no correlation between transthoracic echocardiography (TTE) parameters and BMI was observed. Only LVIDd correlated with disease duration (r = 0.645, p < 0.001). All cardiac functions were within the normal range, indicating no association with functional impairments.Conclusion: Cardiac remodeling in patients with craniopharyngioma correlated with the degree of hypothalamic obesity, disease duration, sex hormone replacement therapy, male gender, and insulin resistance. As echocardiography has limited sensitivity in patients with obesity, further research on more sensitive techniques for cardiac diagnostics in craniopharyngioma patients is warranted.What is Known:•Long-term prognosis in survivors of craniopharyngioma is impaired by obesity and cardiovascular disease.•Associations between echocardiographic findings and clinical and anthropometric parameters after craniopharyngioma are not yet analyzed.What is New:•In patients with childhood-onset craniopharyngioma, cardiac remodeling was associated with hypothalamic obesity, duration of disease, male gender sex hormone replacement, and insulin resistance.•Due to reduced echocardiographic sensitivity caused by obesity-related technical limitations, more sensitive cardiac diagnostics should be considered.

Early diagnosis and post-operative follow-up of 50 Chinese children with craniopharyngioma.

Craniopharyngioma is a relatively common congenital intracranial tumour for children. But only few available studies focused on the endocrine evaluation before diagnosis and post-operative endocrine evaluations of children with craniopharyngioma. This study aims to aid in the early diagnosis of craniopharyngioma (CP) and follow-up post-operative children suffered from craniopharyngioma. Craniopharyngioma patients, as the CP group (n = 50), and healthy children, as the control group (n = 30), the symptoms and pituitary hormone levels were reviewed and investigated. The pre-operative levels of peak of GH, IGF-1, FT4, ACTH, COR and PRL of CP patients were significantly lower than those of the control group (all the P ≤ 0.001). Levels of pituitary-hormones after surgery were significantly lower than both those before surgery and those of the control group (all the P ≤ 0.001). HGH treatment could significantly improve the growth velocity of post-operative children (3.8 ± 1.5 cm/year vs 13.0 ± 3.4 cm/year for males, P ≤ 0.001; 4.0 ± 1.3 cm/year vs 12.7 ± 1.8 cm/year for females, P ≤ 0.001). Children presenting with endocrine disturbance symptoms combined with pituitary hormone deficits should be assessed by MRI to exclude craniopharyngioma earlier. Also, long-term follow-up study was very essential to craniopharyngioma survivors.

Cognitive Performance, Aerobic Fitness, Motor Proficiency, and Brain Function Among Children Newly Diagnosed With Craniopharyngioma.

Craniopharyngioma survivors experience cognitive deficits that negatively impact quality of life. Aerobic fitness is associated with cognitive benefits in typically developing children and physical exercise promotes recovery following brain injury. Accordingly, we investigated cognitive and neural correlates of aerobic fitness in a sample of craniopharyngioma patients. Patients treated for craniopharyngioma [N=104, 10.0±4.6 years, 48% male] participated in fitness, cognitive and fMRI (n=51) assessments following surgery but before proton radiation therapy. Patients demonstrated impaired aerobic fitness [peak oxygen uptake (PKVO2)=23.9±7.1, 41% impaired (i.e., 1.5 SD&lt;normative mean)], motor proficiency [Bruininks-Oseretsky (BOT2)=38.6±9.0, 28% impaired], and executive functions (e.g., WISC-IV Working Memory Index (WMI)=96.0±15.3, 11% impaired). PKVO2 correlated with better executive functions (e.g., WISC-IV WMI r=.27, p=.02) and academic performance (WJ-III Calculation r=.24, p=.04). BOT2 correlated with better attention (e.g., CPT-II omissions r=.26, p=.04) and executive functions (e.g., WISC-IV WMI r=.32, p=.01). Areas of robust neural activation during an n-back task included superior parietal lobule, dorsolateral prefrontal cortex, and middle and superior frontal gyri (p&lt;.05, corrected). Higher network activation was associated with better working memory task performance and better BOT2 (p&lt;.001). Before adjuvant therapy, children with craniopharyngioma demonstrate significantly reduced aerobic fitness, motor proficiency, and working memory. Better aerobic fitness and motor proficiency are associated with better attention and executive functions, as well as greater activation of a well-established working memory network. These findings may help explain differential risk/resiliency with respect to acute cognitive changes that may portend cognitive late effects. (JINS, 2019, 25, 413-425).

Microstructure alterations in the hypothalamus in cranially radiated childhood leukaemia survivors but not in craniopharyngioma patients unaffected by hypothalamic damage.

Metabolic complications are frequent in childhood leukaemia (ALL) survivors treated with cranial radiotherapy (CRT). These complications are potentially mediated by damage to the hypothalamus (HT), as childhood onset (CO) craniopharyngioma (CP) survivors without HT involvement are spared overt obesity. Diffusion tensor imaging (DTI) shows brain tissue microstructure alterations, by fractional anisotrophy (FA), mean diffusivity (MD), axial diffusivity (AD) and radial diffusivity (RD). We used DTI to determine the integrity of the microstructure of the HT in ALL survivors. Case-control study. Three groups were included: (i) 27 CRT treated ALL survivors on hormone supplementation, (ii) 17 CO-CP survivors on hormone supplementation but without HT involvement and (iii) 27 matched controls. DTI parameters of the HT were measured and body composition. Microstructural alterations in the HT were more severe in ALL survivors with a BMI ≥25 than with BMI <25. Compared to controls, ALL survivors had reduced FA (P=.04), increased MD (P<.001), AD (P<.001) and RD (P<.001) in the right and left HT. In the right HT, ALL survivors with a BMI ≥25 showed elevated MD (P=.03) and AD (P=.02) compared to ALL survivors with BMI <25. In contrast, DTI parameters did not differ between CP survivors and controls. Long-term follow-up after CRT for ALL DTI measures were affected in the HT despite complete hormone replacement. The present data suggest that ALL survivors have demyelination and axonal loss in the HT.

Oxytocin in survivors of childhood-onset craniopharyngioma

Quality of survival of childhood-onset craniopharyngioma patients is frequently impaired by hypothalamic involvement or surgical lesions sequelae such as obesity and neuropsychological deficits. Oxytocin, a peptide hormone produced in the hypothalamus and secreted by posterior pituitary gland, plays a major role in regulation of behavior and body composition. In a cross-sectional study, oxytocin saliva concentrations were analyzed in 34 long-term craniopharyngioma survivors with and without hypothalamic involvement or treatment-related damage, recruited in the German Childhood Craniopharyngioma Registry, and in 73 healthy controls, attending the Craniopharyngioma Support Group Meeting 2014. Oxytocin was measured in saliva of craniopharyngioma patients and controls before and after standardized breakfast and associations with gender, body mass index, hypothalamic involvement, diabetes insipidus, and irradiation were analyzed. Patients with preoperative hypothalamic involvement showed similar oxytocin levels compared to patients without hypothalamic involvement and controls. However, patients with surgical hypothalamic lesions grade 1 (anterior hypothalamic area) presented with lower levels (p = 0.017) of oxytocin under fasting condition compared to patients with surgical lesion of posterior hypothalamic areas (grade 2) and patients without hypothalamic lesions (grade 0). Craniopharyngioma patients' changes in oxytocin levels before and after breakfast correlated (p = 0.02) with their body mass index. Craniopharyngioma patients continue to secrete oxytocin, especially when anterior hypothalamic areas are not involved or damaged, but oxytocin shows less variation due to nutrition. Oxytocin supplementation should be explored as a therapeutic option in craniopharyngioma patients with hypothalamic obesity and/or behavioral pathologies due to lesions of specific anterior hypothalamic areas. Clinical trial number: KRANIOPHARYNGEOM 2000/2007(NCT00258453; NCT01272622).

Hypothalamic dysfunction revealed by magnetic resonance diffusion tensor imaging in childhood leukemia survivors treated with cranial radiotherapy but not in craniopharyngioma survivors

Hypothalamic dysfunction revealed by magnetic resonance diffusion tensor imaging in childhood leukemia survivors treated with cranial radiotherapy but not in craniopharyngioma survivors

Craniopharyngioma in Children: Long-term Outcomes.

The survival rate for childhood craniopharyngioma has been improving, with more long-term survivors. Unfortunately it is rare for the patient to be normal, either from the disease itself or from the effects of treatment. Long-term survivors of childhood craniopharyngioma suffer a number of impairments, which include visual loss, endocrinopathy, hypothalamic dysfunction, cerebrovascular problems, neurologic and neurocognitive dysfunction. Pituitary insufficiency is present in almost 100%. Visual and hypothalamic dysfunction is common. There is a high risk of metabolic syndrome and increased risk of cerebrovascular disease, including stroke and Moyamoya syndrome. Cognitive, psychosocial, and emotional problems are prevalent. Finally, there is a higher risk of premature death among survivors of craniopharyngioma, and often this is not from tumor recurrence. It is important to consider craniopharyngioma as a chronic disease. There is no perfect treatment. The treatment has to be tailored to the individual patient to minimize dysfunction caused by tumor and treatments. So “cure” of the tumor does not mean a normal patient. The management of the patient and family needs multidisciplinary evaluation and should involve ophthalmology, endocrinology, neurosurgery, oncology, and psychology. Furthermore, it is also important to address emotional issues and social integration.

Obesity and craniopharyngioma

An epidemic of pediatric obesity has occurred across the world in recent years. There are subgroups within the population at high-risk of becoming obese and especially of having experience of precocious cardiovascular and metabolic co-morbidities of obesity. One of these subgroups comprises patients treated for childhood cancers and namely survivors of craniopharyngioma. The high incidence of obesity in this group makes these patients an important disease model to better understand the metabolic disturbances and the mechanisms of weight gain among cancer survivors. The hypothalamic-pituitary axis damage secondary to cancer therapies or to primary tumor location affect long-term outcomes. Nevertheless, the aetiology of obesity in craniopharyngioma is not yet fully understood. The present review has the aim of summarizing the published data and examining the most accepted mechanisms and main predisposing factors related to weight gain in this particular population.

Health Status in Long-Term Survivors of Pediatric Craniopharyngiomas

Craniopharyngiomas are the third most common pediatric brain tumor and most common pediatric suprasellar tumor. Contemporary treatment of craniopharyngiomas uses limited surgery and radiation in an effort to minimize morbidity, but the long-term health status of patients treated in this fashion has not been well described. The purpose of this study was to analyze the health status of long-term survivors of pediatric craniopharyngioma treated primarily with radiation and conservative surgical resection. Medical records of all long-term survivors of craniopharyngioma treated at St. Jude Children's Research Hospital and then transferred to the long-term follow-up clinic were reviewed. The initial cohort comprised 55 patients. Of these, 51 (93%) were alive at the time of this analysis. The median age at diagnosis was 7.1 years (range, 1.2-17.6 years), and 29 (57%) were male. At the time of analysis, the median survival was 7.6 years (range, 5.0-21.3 years). Diagnosis and treatment included surgical biopsy, resection (n = 50), and radiation therapy (n=48). Only 1 patient received chemotherapy. Polyendocrinopathy was the most common morbidity, with hypothyroidism (96%), adrenocorticotropic hormone deficiency (84%), and diabetes insipidus (53%) occurring most frequently. Half of the patients were hypogonadal, and 33 (65%) were overweight or obese. The most common neurologic problems included shunt dependence (37%), seizures (28%), and headaches (39%). Psychological and educational deficits were also identified in a significant number of these individuals. Despite efforts to reduce morbidity in these patients, many survivors remain burdened with significant medical complications. In a small percentage of patients, complications may result in death even during extended remission of craniopharyngioma. Because of the broad spectrum or morbidities experienced, survivors of craniopharyngioma continue to benefit from multidisciplinary care.

MELATONIN DEFICIENCY AND DISRUPTED CIRCADIAN RHYTHMS IN PEDIATRIC SURVIVORS OF CRANIOPHARYNGIOMA

Craniopharyngiomas are the most common extraneural tumors of the CNS in children.1 Because craniopharyngiomas usually grow along the anatomic midline, the tumors or their treatments (surgical excision and radiation) frequently lead to hypopituitarism, visual field defects, obesity, sleep abnormalities, and daytime hypersomnolence (DH).2,3 DH persists despite hormone replacement or treatment of commonly associated obstructive sleep apnea (OSA). We hypothesized that disrupted sleep patterns in patients with craniopharyngioma result from dysfunction of the hypothalamic circadian pacemaker located in the suprachiasmatic nucleus, which controls the timing of the daily sleep propensity rhythm. Daily variations in levels of the pineal hormone melatonin serve as a marker of the function of this system. Low salivary melatonin has been documented in obese craniopharyngioma survivors and melatonin supplementation has proved beneficial in some cases; however, a detailed analysis of circadian rhythms in this patient population had not been performed.4 ### Methods. Subjects older than 8 years of age with self-reported DH requiring daytime stimulant medication were recruited from a pool of 42 craniopharyngioma survivors treated at the Children’s Hospital Boston (CHB)/Dana-Farber Cancer Institute Brain Tumor Program between 1990 and 2002 by a single neurologist (S.L.P.). Four subjects were enrolled after informed consent was obtained in accordance with the Committee on Clinical Research. Three subjects (2 female, 1 male) ages 15, 15, and 22 completed the study. All had undergone both surgical extirpation and radiotherapy, were morbidly obese (BMI 41–54; normal 17–25), had panhypopituitarism requiring hormone supplementation, and had mild REM-related OSA, and 1 had a seizure disorder. Wrist actigraphy was obtained for 2 to 3 weeks prior to admission …

Predicting behavioral problems in craniopharyngioma survivors after conformal radiation therapy

Although radiation therapy is a primary treatment for craniopharyngioma, it can exacerbate existing problems related to the tumor and pre-irradiation management. Survival is often marked by neurologic deficits, panhypopituitarism, diabetes insipidus, cognitive deficiencies, and behavioral and social problems. The Achenbach Child Behavior Checklist (CBCL) was used to evaluate behavioral and social problems during the first 5 years of follow-up in 27 patients with craniopharyngioma treated with conformal radiation therapy. All group averages for the CBCL scales were within the age-typical range at pre-irradiation baseline. Extent of surgical resection was implicated in baseline differences for the internalizing, externalizing, behavior problem and social scores. Significant longitudinal changes were found in internalizing, externalizing, behavior problem and school scores that correlated with tumor and treatment-related factors. The most common variables implicated in post-irradiation behavioral and social problems were CSF shunting, presence of an Ommaya reservoir, diabetes insipidus, and low pre-irradiation growth hormone levels.

Cardiac Risk After Craniopharyngioma Therapy

Although long-term survival after craniopharyngioma treatment is excellent in childhood and early adulthood, sudden deaths in two craniopharyngioma survivors with cardiac findings suggest a need to determine whether treated patients exhibit potential substrates for sudden cardiac death. We present a retrospective review of two index patients with cardiac mortality. This motivated a prospective cardiac screening of 12 survivors that identified nearly a third with significant QTc prolongation. QTc-prolonging medication and stimulants should be used with caution in this population, and routine electrocardiogram screening may identify those at highest risk.

Secondary narcolepsy may be a causative factor of increased daytime sleepiness in obese patients with childhood craniopharyngioma

Prognosis in childhood craniopharyngioma survivors hinges upon late effects such as pituitary deficiency and obesity. Observations indicate that reduced physical activity and increased daytime sleepiness might be risk factors for obesity. We analyzed the degree of daytime sleepiness in 115 childhood craniopharyngioma patients (47% obese) using the Epworth Sleepiness Scale (ESS). Thirty-five (30%) displayed increased daytime sleepiness (ESS score > 10) of whom 14 were obese (26% of obese cohort). Polysomnography (PSG) and Multiple Sleep Latency Tests (MSLT) were conducted with 10 obese patients presenting increased daytime sleepiness, with only two craniopharyngioma patients revealing a sleep related breathing disorder. Four patients had repeated episodes of SOREM (sleep onset rapid eye movement), the classic PSG criterion for narcolepsy. Three patients displayed hypersomnia. All but one patient qualified as acutely obese. We speculate that secondary narcolepsy is an exacerbating condition of childhood craniopharyngioma obesity, supported by recent reports on orexin and narcolepsy which suggest hypothalamic failure in idiopathic narcolepsy.

Health Related Quality of Life and Psychological Outcome in Patients Treated for Craniopharyngioma in Childhood

Patients with craniopharyngioma are at risk for many adverse effects related to the tumour's invasive behaviour and its proximity to many vital structures. Profound psychosocial problems, memory impairment, pituitary and hypothalamic dysfunction in addition to the physical handicap of visual loss are frequently recognized sequelae of craniopharyngioma treatment. To examine health related quality of life (QoL) and psychological outcomes of patients treated for craniopharyngioma at the Royal Children's Hospital, Melbourne, between January 1980 and September 2003. Seven (17.4%) of 46 (26 male) had died. Thirty-nine remained, of whom 30 were contactable. Eighteen of 30 (8 male), mean age 21.2 +/- 6.7 years, agreed to evaluation, of whom 16/18 (88.9%) had three or more pituitary hormone deficiencies, 11/18 had visual impairment and 9/18 obesity. The Adult GH-Deficient Assessment (AGHDA) and Psychological General Well-Being (PGWB) questionnaires were employed to assess quality of life in patients and age- and sex-matched healthy controls. Additional psychological assessment, including intellectual and academic skills, emotional function, and adaptive behaviour, had been undertaken in 12 patients at a previous time. High levels of physical morbidity and psychological disability were described. The General Health score of patients was significantly worse than for controls on PGWB (p = 0.025), anxiety was higher in those who had surgery alone (p = 0.008) and subjective QoL associated with GHD using AGHDA was lower (p = 0.006). Few craniopharyngioma survivors (18/30) were available for evaluation, demonstrating difficulties in attempts to assess this complex group. The discrepancy between results of objective and subjective measures of QoL is discussed in terms of adaptation to illness, disabilities and changed perception of life fulfilment. Craniopharyngioma and its treatment result in significant, complex medical, social, psychological and emotional difficulties. The degree of global disability is not reflected in subjective QoL reports for this group, highlighting the need for careful selection of assessment instruments.