SARC031: MEK Inhibitor Selumetinib (AZD6244) in Combination With the mTOR Inhibitor Sirolimus for...
Malignant Peripheral Nerve Sheath TumorsNeurofibromatosis 1To determine the clinical benefit rate of selumetinib in combination with sirolimus in patients with unresectable or metastatic neurofibromatosis type 1 (NF1) associated or sporadic MPNST.
SPEARHEAD-3 Pediatric Study
Synovial SarcomaMalignant Peripheral Nerve Sheath Tumor (MPNST)2 moreThis is a pediatric basket study to investigate the safety and efficacy of afamitresgene autoleucel in HLA-A*02 eligible and MAGE-A4 positive subjects aged 2-21 years of age with advanced cancers
Optic Nerve Sheath Diameter in Pediatric Patients
Optic Nerve Sheath NeoplasmIntracranial Pressure Increase2 moreEndotracheal intubation and laryngeal mask are generally applied to secure the airway during general anesthesia. There is a widespread opinion among anesthesiologists that endotracheal intubation increases intracranial pressure. Since there were no non-invasive methods measuring intracranial pressure in the past, adequate studies on this subject could not be done. With this measurement, we aimed to show whether ETT or LMA applications have effects on intracranial pressure.
Multi-parametric Biomarker Development to Predict Malignant Conversion in Patients With Neurofibromatosis...
Neurofibromatosis 1Malignant Peripheral Nerve Sheath Tumor (MPNST) of Soft Tissue (Diagnosis)1 moreThe goal of this prospective observational study is to learn about the utility of imaging and clinical features in patients with Neurofibromatosis type 1 categorized as high risk for the development of malignant peripheral nerve sheath tumors. The main objectives are: To evaluate the prevalence, multi-parametric imaging features of distinct nodular lesions ("DNLs") and natural history in people with NF1 with clinical and genetic features deemed "high-risk" for malignancy. To assess the relationship between individual clinical, genetic and imaging factors that have been suggested to be risk factors for malignant peripheral nerve sheath tumors (MPNST) and the confirmation of atypical neurofibromas (aNF)/ atypical neurofibromatous neoplasm of unknown biologic potential (ANNUBP) or MPNST on pathology. In this research study, the participants will be asked to undergo whole body MRI, provide blood sample and clinical evaluation annually.
Radiographic Prediction of the Nerve Origin of Neck Peripheral Nerve Sheath Tumors
Peripheral Nerve Sheath TumorsSchwannoma of NeckPostoperative nerve palsy is a major complication following resection of neck peripheral nerve sheath tumors. This study is a retrospective cohort aimed to predict the nerve origin of neck peripheral nerve sheath tumors. Accurate preoperative identification of the nerve origin can improve surgical outcomes and patient counseling
Multi-Institutional Registry for Malignant Peripheral Nerve Sheath Tumors
Malignant Peripheral Nerve Sheath TumorsFirst, the investigators plan to use a retrospective analysis to determine the clinical landscape of neurofibromatosis (NF)1-associated malignant peripheral nerve sheath tumor (MPNST) and precursor lesions (e.g., atypical or nodular plexiform neurofibromas). A worldwide database will be established, collecting, in a standardized manner, histologic, immunohistochemical, molecular, radiographic, treatment, and related clinical data from centers worldwide with expertise in these NF1-related cancers. Although retrospective in nature, the resulting data from this registry may reveal previously unanticipated patterns, similar to the INFACT effort outcome. This registry would then allow the acquisition of data associated with MPNST biospecimens collected under associated banks (frozen or paraffin-embedded, germline (or normal tissue DNA) samples, and any previously somatic whole-exome or whole-genome sequencing data for aggregate analyses). Second, the investigators plan to co-register patients to institutional banks in order to prospectively collect MPNST samples for analysis. These patients will be consented in order to collect the above information and for banking of tumor tissue and future studies that include genomic characterization of the tumors.
Proton Therapy Research Infrastructure- ProTRAIT- Neuro-oncology
AstrocytomaEpendymoma10 moreThe 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.
Imatinib Mesylate Treatment of Patients With Malignant Peripheral Nerve Sheath Tumors
Malignant Peripheral Nerve Sheath TumorsThis study assesses the safety and efficacy of imatinib mesylate treatment of patients with malignant peripheral nerve sheath tumors
Sapanisertib or Pazopanib Hydrochloride in Treating Patients With Locally Advanced or Metastatic...
High Grade SarcomaMetastatic Leiomyosarcoma17 moreThis phase I/II trial studies the side effects and best dose of sapanisertib and to see how well it works compared to pazopanib hydrochloride in treating patients with sarcoma that is too large to be removed (locally advanced) or has spread to other areas of the body (metastatic). Sapanisertib and pazopanib hydrochloride may stop the growth of tumor cells by blocking some of the enzymes needed for cell growth.
A Study of Pembrolizumab in Patients With Malignant Peripheral Nerve Sheath Tumor (MPNST), Not Eligible...
Malignant Peripheral Nerve Sheath Tumour (MPNST)This is phase II, single arm, open-label, interventional trial of pembrolizumab (MK-3475) in subjects with metastatic or locally advanced/unresectable or metastatic malignant peripheral nerve sheath tumour (MPNST). The patients will be treated with pembrolizumab for up to 10 cycles. Primary objective is to evaluate the percentage of patients with curatively unresectable MPNST who have achieved clinical response; complete response (CR), partial response (PR), or stable disease (SD) at 18 weeks as assessed by the Investigator, by using RECIST, v1.1.