Nutritional and Metabolic Diseases

Symptoms and General Pathology

All Conditions

Neoplasms

Nervous System Diseases

Musculoskeletal Diseases

Behaviors and Mental Disorders

Rare Diseases

Obesity

Brain Neoplasms

Craniopharyngioma

Adamantinoma

Ameloblastoma

Neuroectodermal Tumors

Neuroectodermal Tumors, Primitive

Neoplasms, Germ Cell and Embryonal

Neoplasms by Histologic Type

Neoplasms, Nerve Tissue

Bone Neoplasms

Bone Diseases

Quality of Life

Brain Tumor, Childhood

Neuroepithelioma

Children and adolescents \>= 18 years of age new diagnosed with craniopharyngioma

* \>= 5 years of age and with incomplete resected tumor =\> randomized in two arms: immediate irradiation after surgery

incomplete resection, wait and watch, MRI-controls every 3 months, and irradiation at the time of progression of residual tumor

Immediate irradiation after incomplete resection (fractionated, percutaneous irradiation, 54 gy)

Radiation

wait ans watch, MRI controls, irradiation at the time of progression of residual tumor

wait and watch

Hermann L Müller, MD

The present investigation is a prospective, multicenter study evaluating craniopharyngioma patients' prognoses following the various currently-practiced therapeutic strategies.Primary goals of the study are to establish quality standards and compare the various therapy strategies with respect to their effectiveness and impact on the quality of life of treated patients. A stratified randomization of two treatment arms will be conducted with respect to timing of postoperative irradiation for the subgroup of patients ≥5 years of age whose tumors are incompletely resected. The researchers will investigate whether an immediate, postoperative irradiation is superior to progression-contingent irradiation based on alterations to quality of life (PEDQOL) from the time randomization is initiated (3rd month post op) to 3 years after randomization. Progression-free survival and overall survival will be examined as closely-related subgoals.Postoperative data will be evaluated via a surveillance study for all complete resection patients as well as for those patients under 5 years of age regardless of their resection grade.

Prospective Study of Children and Adolescents With Craniopharyngioma

KRANIOPHARYNGEOM 2007 - Multicenter Prospective Study of Children and Adolescents With Craniopharyngioma

•Randomized investigation of children (≥ 5 years of age) and adolescent QoL changes (main goal assessment) following incomplete craniopharyngioma resections at primary diagnosis per QoL endpoint scores (PEDQOL "physical functions" domain) measured from 3rd month after surgery for 3 years after randomization; and the progression-free and total survival rates (subgoal assessments) relative to how these rates relate to postoperative irradiation timing (immediate postsurgery irradiation versus wait-and-see approach + progression-contingent irradiation of residual tumor).

* Compilation of applied therapy strategies for craniopharyngiomas in children and adolescents using data capture conforming to SIOP brain tumor groups
* Evaluation of patients' remission status following the various therapy strategies/modalities for craniopharyngiomas using data detection consistent with SIOP brain tumor groups
* Evaluation of the health status (ophthalmologic, neuropsychologic and endocrine conditions) and health-related QoL of children and adolescents following treatment of craniopharyngiomas using data capture conforming to SIOP brain tumor groups

German Society for Pediatric Oncology and Hematology GPOH gGmbH

Arbeitsgemeinschaft Pädiatrische Endokrinologie (APE)

Deutsche Gesellschaft für Endokrinologie (DGE)

Arbeitsgemeinschaft Pädiatrische Radioonkologie (APRO)

Deutsche Gesellschafr für Radioonkologie (DEGRO)

Deutsche Kinderkrebsstiftung

International Society of Paediatric Oncology

Klinikum Oldenburg gGmbH

Professor MD

Cognitive Dysfunction

Cognition Disorders

Central Nervous System Neoplasms

Nervous System Neoplasms

Brain Diseases

Central Nervous System Diseases

* Prior to surgery, subjects will have a complete standard of care history and physical exam by the neurosurgeon and then undergo peri-diagnostic neurocognitive testing utilizing the NIH Toolbox Cognitive Battery computer testing software for iPad (will take 45 minutes). The peri-diagnostic period will be defined as the period from first presentation to 2 weeks post-diagnosis or two weeks post-op, whichever is later.
* Participants will also undergo a rsfcMRI during their standard of care MRI imaging which will add 15 minutes to their scan time

* Patients with diagnosis of a posterior tumor will undergo neurocognitive testing utilizing the NIH Toolbox. Testing will be every 6 months for children currently receiving therapy and annually for those that have completed all therapy for a total of 3 sessions. During standard of care MRI images, rsfcMRI imaging will be performed which will add 15 minutes of time. rsfcMRI will be obtained every 6 months for children currently receiving therapy and annually for those that have completed therapy for a total of 3 rsfcMRIs for each patient.
* Patients in Cohort 1 will also be eligible to continue with the longitudinal assessment as just described. These participants will then undergo repeat neurocognitive testing using the NIH toolbox 6-9 months for an additional 3 testing sessions. rsfcMRI will be obtained during their follow-up imaging at 6-9 month intervals for a total of 3 additional rsfcMRIs (4 total scans).

* During standard of care MRI images, rsfcMRI imaging will be performed which will add 15 minutes of scan time.
* rsfcMRI will be obtained annually for total of 3 rsfcMRIs for each patient.

Undergo the following MRI images: RSFC MRI, T2/T1 MRI, and DTI.

* Will complete cognitive testing using the NIH Toolbox Cognitive Battery
* All patients will complete the Attention Deficit/Hyperactivity Disorder (ADHD) Rating Scale - 5 questionnaire. All patients will complete a self-reported assessment of neurological quality of life (QOL). Adult patients (age ≥ 18 years) will complete the adult version of the Quality of Life in Neurological Disorders (Neuro-QOL) Cognitive Function measure. All pediatric patients will complete the pediatric version of the Neuro-QOL Cognitive Function measure. For patients \<18 years of age and for adult patients with parent present, parent/caregiver will complete the PROMIS® Parent Proxy for cognition. All adult patients will complete the Colorado Learning Difficulties Questionnaire (CLDQ). This questionnaire will be completed by parent/caregiver for patients \< 18 years of age.

* During standard of care MRI images, rsfcMRI imaging will be performed which will add 15 minutes of scan time.
* Will complete cognitive testing using the NIH Toolbox Cognitive Battery
* All patients will complete the Attention Deficit/Hyperactivity Disorder (ADHD) Rating Scale - 5 questionnaire. All patients will complete a self-reported assessment of neurological quality of life (QOL). Adult patients (age ≥ 18 years) will complete the adult version of the Quality of Life in Neurological Disorders (Neuro-QOL) Cognitive Function measure. All pediatric patients will complete the pediatric version of the Neuro-QOL Cognitive Function measure. For patients \<18 years of age and for adult patients with parent present, parent/caregiver will complete the PROMIS® Parent Proxy for cognition. All adult patients will complete the Colorado Learning Difficulties Questionnaire (CLDQ). This questionnaire will be completed by parent/caregiver for patients \< 18 years of age.

Neurocognitive testing

The imaging protocols will be a combined version of a standard of care (SOC) protocol with a research portion. The SOC portion of the pre-surgical scans includes a 3D T2-weighted (T2w) scans and 3D pre- and post-contrast T1-weighted (T1w) images, both at high resolution (1.0 mm3) for use in the intraoperative navigation system. This scan also includes a DTI portion for definition of fiber bundles of interest. The SOC portion of the post-surgical scans includes transverse FLAIR and post-contrast T1w images in all 3 planes. The research portion of the scan will be identical at all 3 time points and include 15 min of rsfcMRI during which the non-sedated subjects will be asked to stay still and awake while looking at a fixation cross

rsfcMRI

Stephanie Perkins, M.D.

Scott Marek, Ph.D.

David Limbrick, M.D., Ph.D.

Joshua Shimony, M.D., Ph.D.

Josh Rubin, M.D., Ph.D.

Dennis Barbour, M.D., Ph.D.

The investigators will focus on three cohorts of brain tumor patients aged, 4-18 years, to answer two critical questions: 1) Can the investigators acquire high quality data relevant to cognitive function during the peri-diagnostic period and, 2) can the investigators develop predictive models for cognitive outcomes using serial examination of functional imaging and cognitive function. Any patient with a newly diagnosed brain tumor aged 4-18 will be eligible for enrollment in cohort 1. Only patients with previously diagnosed tumors of the posterior fossa will be eligible for cohort 2. For cohort 3, eligible patients will include patients with a clinical diagnosis of posterior fossa syndrome with physical impairments that prohibit completion of the NIH Toolbox Cognitive Battery. The investigators have decided to expand the eligible tumor types to better capture the most significant deficit variability that can be caused by tumors outside the posterior fossa. Thus, this focus will provide a platform to analyze the impact that different tumor types and different standard treatments have on cognitive dysfunction. The rationale for inclusion of subjects on cohort 3 is that posterior fossa syndrome is one of the most cognitively devastating diagnoses following a posterior fossa surgery. The causes of posterior fossa syndrome and unknown and there are currently no interventions to improve symptoms. RsfcMRI would offer a novel and non-invasive assessment of posterior fossa syndrome patients by assessing connectivity within and outside of the cerebellum. Expanding the tumor eligibility will allow us to further explore the effect tumor location will have on cognitive testing and rsfcMRI. Here, repeated evaluations on and off therapy will provide the necessary data points to establish trajectories of cognitive development and recovery in this population.

Cognitive Biomarkers in Pediatric Brain Tumor Patients

As dictated by information theory, properly combining predictors across modalities must increase the accuracy of the prediction algorithm, unless a given modality contains no information about the question of interest (in this case, long-term cognitive outcomes).

Children's Discovery Institute

Neurosurgery Research & Education Foundation

The Andrew McDonough B+ (Be Positive) Foundation

Washington University School of Medicine

Gland and Hormone Related Diseases

Diseases and Abnormalities at or Before Birth

Adenoma

Pituitary Diseases

Cysts

Meningioma

Arachnoid Cysts

Pituitary Neoplasms

Neoplasms, Glandular and Epithelial

Hypothalamic Diseases

Endocrine System Diseases

Neoplasms, Vascular Tissue

Meningeal Neoplasms

Endocrine Gland Neoplasms

Hypothalamic Neoplasms

Supratentorial Neoplasms

Central Nervous System Cysts

Congenital Abnormalities

Nervous System Malformations

Lipogranulomatosis

Hermann L Mueller, MD

Svenja Boekhoff

KRANIOPHARYNGEOM Registry 2019 will prospectively collect and descriptively analyse data on diagnostics, treatment, and follow-up of patients with craniopharyngioma. In continuation of preceding studies also patients with xanthogranuloma, meningioma, pituitary adenoma, prolactinoma and cystic intracranial malformations will be registered.

Multicenter Registry for Patients With Childhood.Onset Craniopharyngioma, Xanthogranuloma, Cysts of Rathke's Pouch, Meningioma, Pituitary Adenoma, Arachnoid Cysts

KRANIOPHARYNGEOM Registry 2019 Multicenter Registry for Patients With Childhood-onset Craniopharyngioma, Xanthogranuloma, Cysts of Rathke's Pouch, Meningioma, Pituitary Adenoma, Arachnoid Cysts

Overall survival as measured by Kaplan Meyer analyses

Progression-free survival as measured by Kaplan Meyer analyses

Domains of the PedQol instrument (questionnaire for assessment of health-related QoL in childhood cancer patients.

Deutsche Gesellschaft für Kinderendokrinologie und Diabetologie (DGKED)

Deutschen Gesellschaft für Radioonkologie (DEGRO)

A prospectively recruited of newly-diagnosed pediatric/adolescent patients with brain tumors or head/neck cancers

standard adjuvant local radiation therapy or cranial radiation therapy

Chi-Cheng Yang, Ph.D.

Shinn-Yn Lin, M.D.

Background: Neurobehavioral functions and quality of life (QoL) are the important outcome measurements after radiotherapy in patients with brain tumors and even head/neck cancers. However, few studies have focused on neurobehavioral functions and QoL after anti-cancer treatment particularly brain radiotherapy for pediatric/adolescent patients with brain tumors. This study thus aims to prospectively evaluate those functions in pediatric or adolescent patients with brain or head/neck tumors in order to provide useful information about their clinical outcomes.

Methods: A total of 72 pediatric/adolescent patients, who are diagnosed with brain tumors or head/neck cancers, were prospectively recruited. Neurobehavioral functions will be evaluated using a neuropsychological battery, which includes general cognitive functions, intelligence, memory, executive functions, information processing and emotional/behavioral expressions. The QoL will be evaluated by the health-related QoL questionnaire. All participants will be examined at six phases, which include pre-treatment, 1-month post-treatment, 4-month post-treatment, 1-year post-treatment, 2-year post-treatment and 3-year post-treatment.

Expected results: Patients'neurobehavioral functions and QoL will show significant improvement after treatment, and the improvement will not be diminished across each post-treatment phase.

Neurobehavioral Outcomes and Quality of Life in Pediatric Patients With Brain or Head/Neck Tumors Receiving Proton or Photon Radiotherapy

Intellectual functioning mainly includes subtests of Wechsler Intelligence scale for Children

In this study, evaluations of neurocognitive functions include memory scores of verbal memory (NEPSY-II),memory scores of non-verbal memory (NEPSY-II) raw scores of executive functions (NEPSY-II), raw scores of processing speed (NEPSY-II), raw scores of attention (Continuous Performance Test, CPT).

Evaluations of psychosocial adjustments mainly include severity scores of behavioral problems (Achenbach System of Empirically Based Assessment),scores of behavioral problems(Vineland Adaptive Behavior Scale), scores of parental stress (Parenting Stress Index, PSI) and scores on the scale for quality of life (Child Health Questionnaire, CHQ).

Chang Gung Memorial Hospital

Other Dietary Supplements

All Drugs and Chemicals

Pyruvate

Patients will receive a one-time injection of Hyperpolarized Pyruvate prior to a single MR imaging examination that includes the acquisition of HP carbon-13 metabolic data.

Hyperpolarized Pyruvate

Sabine Mueller, MD, PhD, MAS

This is a single arm pilot trial within the Pacific Pediatric Neuro-Oncology Consortium (PNOC).

The pilot study will look at the safety and toxicity of acquiring hyperpolarized carbon-13 imaging in children with brain tumors.

This is an open label trial to assess the safety and tolerability of the adult tolerated dose of Hyperpolarized Pyruvate (HP) for metabolic imaging in children with brain tumors who do not require sedation for their MR imaging. Nine patients will receive a single MR imaging examination that includes the acquisition of hyperpolarized 13C metabolic data in combination with anatomic, diffusion, perfusion and lactate edited 1H spectroscopic imaging data. The data will be processed using custom designed software to estimate changes in levels of lactate/pyruvate and to relate them to abnormalities observed in the data from other MR modalities.

The results of this study will provide the safety data required to move this type of metabolic imaging into therapeutic trials to assess the utility of HP 13C lactate/pyruvate as a new surrogate marker of drug tumor penetration and early response to therapy in children with brain tumors.

Pilot Study of Safety and Toxicity of Acquiring Hyperpolarized Carbon-13 Imaging in Children With Brain Tumors

PNOC 011: Pilot Study of Safety and Toxicity of Acquiring Hyperpolarized Carbon-13 Imaging in Children With Brain Tumors

Dose Limiting Toxicities will be assessed by monitoring for adverse events, scheduled laboratory assessments, vital sign measurements, ECGs, and physical examinations. The severity of the toxicities will be graded according to the NCI CTCAE v4.0. Adverse events and clinically significant laboratory abnormalities will be summarized by maximum intensity and relationship to study drug. Safety will be assessed during the infusion and at least for one hour after completion of the infusion as well as by phone 24 hours after the infusion. Descriptive statistics will be utilized to display the data on toxicity seen. Analyses will be performed for all patients having received at least one dose of study drug.

13C data will be acquired using the following sequence parameters;

* a volumetric acquisition from a 4-5cm slice with 2D phase encoding and 1-D Echo-Planar Spectroscopic Imaging (EPSI) encoding, field of view (FOV) 20x24x10cm (1cc voxel size),
* 1H scout; A further set of scout images will be obtained to re-establish appropriate landmarks.
* High resolution anatomic imaging: These require a 3D localizing scan to define the graphical prescription;
* Diffusion Images: This will be followed by diffusion tensor spin echo single shot echo planar images, 6 gradient directions, 22cm FOV, 128x128 matrix,
* Lactate edited 3D magnetic resonance spectroscopic imaging (MRSI): water suppressed 1H magnetic resonance spectroscopy (MRS) with PRESS volume selection, out-of-voxel suppression with very spatially selective rf pulses, echo planar encoding in the signal intensity (SI) direction and 2D in-plane phase encoding

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization
Prospective Study of Children and Adolescents With Craniopharyngioma	NCT01272622	Craniopharyngio... Obesity	Radiation wait and watch	1 Day - 18 Years	Klinikum Oldenburg gGmbH
Cognitive Biomarkers in Pediatric Brain Tumor Patients	NCT02914067	Childhood Brain...	Neurocognitive ... rsfcMRI	4 Years - 18 Years	Washington University School of Medicine
Cognitive Biomarkers in Pediatric Brain Tumor Patients	NCT02914067	Childhood Brain...	Neurocognitive ... rsfcMRI	4 Years - 18 Years	Washington University School of Medicine
Prospective Study of Children and Adolescents With Craniopharyngioma	NCT01272622	Craniopharyngio... Obesity	Radiation wait and watch	1 Day - 18 Years	Klinikum Oldenburg gGmbH
Multicenter Registry for Patients With Childhood.Onset Craniopharyngioma, Xanthogranuloma, Cysts of Rathke's Pouch, Meningioma, Pituitary Adenoma, Arachnoid Cysts	NCT04158284	Craniopharyngio... Obesity		1 Day - 18 Years	Klinikum Oldenburg gGmbH
Cognitive Biomarkers in Pediatric Brain Tumor Patients	NCT02914067	Childhood Brain...	Neurocognitive ... rsfcMRI	4 Years - 18 Years	Washington University School of Medicine
Neurobehavioral Outcomes and Quality of Life in Pediatric Patients With Brain or Head/Neck Tumors Receiving Proton or Photon Radiotherapy	NCT02608762	Brain Tumor	standard adjuva...	1 Month - 18 Years	Chang Gung Memorial Hospital
Multicenter Registry for Patients With Childhood.Onset Craniopharyngioma, Xanthogranuloma, Cysts of Rathke's Pouch, Meningioma, Pituitary Adenoma, Arachnoid Cysts	NCT04158284	Craniopharyngio... Obesity		1 Day - 18 Years	Klinikum Oldenburg gGmbH
Pilot Study of Safety and Toxicity of Acquiring Hyperpolarized Carbon-13 Imaging in Children With Brain Tumors	NCT02947373	Pediatric Brain...	Hyperpolarized ...	3 Years - 18 Years	University of California, San Francisco

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