This trial is evaluating whether Focused Ultrasound with neuro-navigator-controlled sonication will improve 1 primary outcome and 3 secondary outcomes in patients with Diffuse Intrinsic Pontine Glioma. Measurement will happen over the course of Up to 6 months after last FUS treatment..
This trial requires 3 total participants across 2 different treatment groups
This trial involves 2 different treatments. Focused Ultrasound With Neuro-navigator-controlled Sonication is the primary treatment being studied. Participants will all receive the same treatment. There is no placebo group. The treatments being tested are in Phase 1 and are in the first stage of evaluation with people.
In most patients with diffuse intrinsic pontine glioma, surgical resection of the tumor is indicated, but often the patient does not have a long-term prognostic advantage from this treatment. Radiochemotherapy is often used as first-line treatment. At the Mayo Clinic, this treatment has been associated with improvement in survival, but this result must be viewed with caution because most of the patients who had radiochemotherapy have had chemotherapy before and radiation therapy after.
This analysis is one of the largest cohorts of DAI patients reported in the literature. While overall survival is excellent, the median overall survival in newly diagnosed DAI is less than 6 months.
Symptoms can be variable. A mass effect on the brainstem should be ruled out. The extent of tumor should be assessed before treatment can be proposed. Early diagnosis of diffuse intrinsic pontine glioma is associated with good prognosis. Imaging at presentation (MRI) may be used for diagnosis. Patients should be considered at risk and be referred to a high-grade glioma/meningioma center. Diffuse intrinsic pontine glioma is a very aggressive disease with a long remission interval of years following initial treatment. Early diagnosis can impact the number of patient-related treatment and outcomes.
Most children with DIPG who will die without signs of tumor recurrence have very good initial survival, particularly in cases with low-grade or asymptomatic disease. Long-term survival is poor in patients with high grade disease.
The exact cause of diffuse intrinsic pontine glioma is unknown. Inherited genetic factors may play a significant role in developing diffuse intrinsic pontine glioma. Diffuse intrinsic Pontine Glioma (DIPG) may result from mutations in one of the genes of the Hedgehog pathway.
The most common symptoms of diffuse intrinsic pontine glioma are headache and visual impairment. A seizure diagnosis is the most common incidental finding in the presence of diffuse intrinsic pontine glioma. However, the seizure was a nonspecific indicator of diffuse intrinsic pontine glioma. Seizures can be caused by many conditions, and therefore a seizure diagnosis does not reliably indicate diffuse intrinsic pontine glioma. Tumor-associated ophthalmoplegia can happen in diffuse intrinsic pontine glioma, but the diagnosis can be supported by tumor cells in the orbital tissue. Tumor-associated ophthalmoplegia is a nonspecific presentation of diffuse intrinsic pontine glioma.
This preliminary study suggests that focused ultrasound with SNC-NVS is safe for people with or without metal implants or implants in the brain tissue and does show a good feasibility and tolerability. More research is needed to confirm these findings and to extend the follow-up period.
Recent findings suggests that DIPG may be under-diagnosed and, more than likely, some cases may simply go undiagnosed. The number of cases of DIPG currently diagnosed is likely greater than the number of cases which have been missed. There is a need to educate the public and healthcare professionals and to implement national initiatives to increase awareness of the disease and to support early diagnosis and appropriate treatment.
The risk for the development of DIPG over an average of 4 years is approximately 4/100,000 for women and less than 0.001 for men. These values can be used to counsel patients and their families about the risk.
It is a rare event for PGG to be caused by a primary brainstem glioma. Although the tumor was not surgically biopsy-proven, histologic findings, tumor localization, and clinical presentation strongly support PGG as a primary brainstem glioma. These lesions are typically treated with radiation therapy.
This report indicates the efficacy and safety of NNC sonication with focussed ultrasound. The improvement of PVS in a clinical study, using NNC sonication in combination with FUS, warrants further study.
This technique is useful to reduce the number of inlets to the tumor and to identify the best one. However, there were a lot of malpositioned inlets inside the tumor as well as other inelinities nearby which makes the ultrasound ineffective.