View clinical trials related to Optic Nerve Glioma.
Filter by:Infantile optic pathway glioma (OPG) is generally benign and slow-growing, but due to infiltration and compression of sensitive neuronal structures in the optical pathways, progressive visual loss is a frequent and highly debilitating complication of the condition. Recently, therapeutic strategies aimed at neuroprotection in the visual pathway rather than reducing the size of the tumor have been studied. Nerve growth factor (NGF) is a neurotrophin that acts on peripheral and central neurons by binding with high affinity to the trkANGFR receptor, which has tyrosine kinase activity, and with low affinity to the non-selective pan-neurotrophin receptor p75NTR that regulates signaling through trkANGFR. The effect of NGF on target cells depends on the ratio of these two co-distributed receptors on the cell surface. Recently, two studies have shown that murine NGF can prevent progression of visual damage in OPG patients. These successful exploratory studies (the last of which was a randomized, double-blind, placebo-controlled study) represent a significant reference point in the field of vision loss in OPG patients and provide the basis and rationale for this study using a recombinant form of mutated NGF, painless NGF (CHF6467), which is anticipated to prove devoid of adverse effects related to pain at therapeutic doses. The purpose of this randomised study is to assess the safety and efficacy of multiple doses of painless NGF CHF6467 eye drops on the visual function of children or young adults with optic pathway gliomas, whether or not associated with type 1 neurofibromatosis. This study will include serial assessments of both optical pathway functionality and morphology, using electrophysiological and magnetic resonance imaging (MRI) techniques of the brain. The comparator will be a placebo preparation based on a physiologically balanced salt solution. This comparator has no effect on retinal function and optic nerve, is painless and perfectly tolerated, as reported by numerous clinical studies including that of our group.
Optic pathway glioma (OPG) can result in visual deterioration. Symptomatic patients often report deficits in visual acuity (VA), visual field, visual-evoked potentials (VEPs), strabismus, proptosis, disc swelling, and other visual/neurological problems. VA itself remains one of the most important outcome measures for OPG patients, with various studies showing strong ties of VA level to overall quality of life and well-being . Maintenance of favorable VA and vision outcomes is of paramount importance in the management of OPG. In terms of management of OPG, surgery and radiotherapy are used on a more limited basis because of location of the tumors and risk of secondary tumors, respectively. Tumor stabilization often prioritized, and chemotherapy is considered ideal for tumor stabilization in OPG, but vision is not always retained and may worsen in some cases, partially due to low radiographic efficacy and long time interval to response of the current chemotherapy regimen. In the prior study, the investigators modified the traditional carboplatin combined with vincristine regimen by increasing the dose of carboplatin and combining with an anti-angiogenic drug. Of the 15 OPG patients, objective response rate was 80% and the time to response was only 3.3 months. 8 (53%) patients experienced an improvement in visual acuity during therapy and 6 (40%) were stable, which was higher than the historical studies. This study was launched to further verify the clinical efficacy of the modified regimen and its effect on visual acuity improvement.
The first proton therapy treatments in the Netherlands have taken place in 2018. Due to the physical properties of protons, proton therapy has tremendous potential to reduce the radiation dose to the healthy, tumour-surrounding tissues. In turn, this leads to less radiation-induced complications, and a decrease in the formation of secondary tumours. The Netherlands has spearheaded the development of the model-based approach (MBA) for the selection of patients for proton therapy when applied to prevent radiation-induced complications. In MBA, a pre-treatment in-silico planning study is done, comparing proton and photon treatment plans in each individual patient, to determine (1) whether there is a significant difference in dose in the relevant organs at risk (ΔDose), and (2) whether this dose difference translates into an expected clinical benefit in terms of NormalTissue Complication Probabilities (ΔNTCP). To translate ΔDose into ΔNTCP, NTCP-models are used, which are prediction models describing the relation between dose parameters and the likelihood of radiation-induced complications. The Dutch Society for Radiotherapy and Oncology (NVRO) setup the selection criteria for proton therapy in 2015, taking into account toxicity and NTCP. However, NTCP-models can be affected by changes in the irradiation technique. Therefore, it is paramount to continuously update and validate these NTCP-models in subsequent patient cohorts treated with new techniques. In ProTRAIT, a Findable, Accessible, Interoperable and Reusable (FAIR)data infrastructure for both clinical and 3D image and 3D dose information has been developed and deployed for proton therapy in the Netherlands. It allows for a prospective, standardized, multi-centric data from all Dutch proton and a representative group of photon therapy patients.
Phase I and II study of the MEK inhibitor Selumetinib given twice daily on 5 out of 7 days in children with NF1 and inoperable plexiform neurofibromas or progressive/relapsed optic pathway gliomas. This study will test the early and late toxicities of selumetinib when it is given in this intermittent schedule (in 5 out of 7 days) and will also test the effectiveness of the drug in reducing the size of plexiform neurofibromas and optic pathway gliomas in children with NF1. It will also test the effectiveness of the drug in improving the participants function in day to day life.
The investigators hypothesize that this study will show that sufficient lymphocyte stem cell can be harvested prior chemoradiation and be reinfused back after treatment, and at least 5 of the 10 patients (50%) will achieve an absolute increase of lymphocyte counts of 300 cells/mm^3 four weeks after stem cell reinfusion in high grade glioma patients.
This pilot phase II trial studies how well selumetinib works in treating patients with neurofibromatosis type 1 and cutaneous neurofibromas. Selumetinib may stop the growth of tumor cells by blocking some of the enzymes needed for cell growth.
This phase I trial studies the side effects and best dose of entinostat in treating pediatric patients with solid tumors that have come back or have not responded to treatment. Entinostat may block some of the enzymes needed for cell division and it may help to kill tumor cells.
By employing a combination of advanced MRI techniques and correlative serum biomarkers of blood brain barrier (BBB) disruption, the investigators plan to develop a powerful, first of its kind clinical algorithm in pediatrics whereby the investigators can measure and identify the window of maximal BBB disruption post MLA to 1) allow for an alternative to surgery in incompletely resected tumors, 2) allow for optimal chemotherapeutic dosing to achieve the greatest benefits and the least systemic side effects and 3) distinguish subsequent tumor progression from long-term MLA treatment effects. Preliminary data in adult imaging studies have shown that the BBB disruption lasts for several weeks following treatment before returning to a low baseline. This pilot therapeutic study will provide preliminary validation in pediatric patients.
This is a phase II study of the drug, pegylated interferon alfa-2b (PEG-Intron), used to treat brain tumors in a pediatric population. Researchers want to see if treatment with PEG-Intron will stop tumor growth for patients with juvenile pilocytic astrocytomas or optic pathway gliomas. The purposes of this study are: - To learn more about the response to pegylated interferon - To learn more about the side effects of pegylated interferon - To learn more about MRI images in patients with Juvenile Pilocytic Astrocytomas or Optic Pathway Gliomas. - To learn more about quality of life in patients treated with pegylated interferon
This pilot clinical trial studies gallium Ga 68-edotreotide (68Ga-DOTATOC) positron emission tomography (PET)/computed tomography (CT) in finding brain tumors in younger patients. Diagnostic procedures, such as gallium Ga 68-edotreotide PET/CT imaging, may help find and diagnose brain tumors.