Transformation of Plexiform Neurofibromas to Malignant Peripheral Nerve Sheath Tumors in Neurofibromatosis...
NeurofibromatosisMPNSTBackground:<TAB> - Many people with neurofibromatosis type 1 (NF1) get tumors of the nervous system. Finding malignant tumors early is important for removing them. Researchers want to find ways of doing this with scans and genetic testing. Objectives: - To learn more about neurofibromatosis type 1. Eligibility: - People age 10 and older with NF1 who have a benign tumor or have had a malignant one. Design: Participants will be screened in another study with medical history, physical exam, and urine and blood tests. They will have a magnetic resonance imaging (MRI) scan. MRI: Participants will lie on a table that slides into a metal cylinder. They will be in the scanner for 60 90 minutes, lying still for 15 minutes at a time. Participants will get earplugs for the loud sounds. They will get a contrast agent (dye) through a thin plastic tube (catheter) inserted in an arm vein. As part of their regular care, participants will have: FDG-PET/CT scan. They will get radioactive glucose (sugar) through a catheter in an arm vein. [18F]-FLT-PET/CT scan. This is like the FDG scan but with a different radioactive chemical. Biopsy. A piece of tumor tissue is removed with a needle. A piece of tissue from a previous biopsy may also be studied. Participants may have genetic testing. Blood will be taken. It will be tested along with biopsy samples. Researchers will explain the risks and procedures. They may notify participants if testing shows health problems. After this study, participants will continue their regular cancer care.
Incidence of Malignant Peripheral Nerve Sheath Tumor (MPNST) Development in Participants With Neurofibromatosis...
Neurofibromatosis 1Peripheral Nerve Neoplasms1 moreBackground: NF1 is a genetic syndrome. Tumors appear early in life. Many people with NF1 develop PN. These tumors can become an aggressive cancer called MPNST. People with MPNST may benefit from treatment with a MEK inhibitor (MEKi). Researchers want to learn if there is an increased risk of MPNST formation from MEKi treatment in people with NF1. To do this, they will review data that has been collected in NIH NF1 studies. Objective: To describe the characteristics of people who have taken part in NF1 studies at NIH and to compare the risk of MPNST formation in those treated with MEKi or other PN-directed treatment. Eligibility: People with NF1 who were seen at NIH from Jan. 1, 1998, to Jan. 1, 2020. Design: Participants medical records will be reviewed. Participants who opted out of future use of their data will not be included. Demographic data, like sex, race, and date of birth, will be collected. Data about MEKi and non-MEKi treatments will be collected. Clinical data, such as surgery and treatment details, will be collected. The differences between all participants who were seen at NIH for any NF1 related study will be compared. Participants will be put into 4 groups: History of MEKi therapy Treatment with tumor directed therapy other than MEKi Treatment with both MEKi and non-MEKi tumor directed therapies No tumor directed medical therapy Participants with NF1 who were treated for PN with either a MEKi treatment or a non-MEKi treatment will also be compared. The study will last for 3 to 6 months.
Expanded Access for DeltaRex-G for Advanced Pancreatic Cancer, Osteosarcoma, Soft Tissue Sarcoma...
Pancreatic CancerOsteosarcoma6 moreForty patients with advanced pancreatic cancer, osteosarcoma, soft tissue sarcoma, and breast cancer will receive DeltaRex-G intravenously at a dose of 1-4 x 10e11 colony forming units (cfu) or equivalent 0.6-1.8 x 10e10 RV copies per dose one to three times a week. DeltaRex-G may or may not be given with one or more FDA approved cancer therapies/immunotherapies. Based on previous Phase 1/2 US based clinical studies, DeltaRex-G does not suppress the bone marrow or cause serious organ dysfunction, and enhanced immune cell trafficking in tumors may cause the tumors to appear larger or new lesions to appear on CT, PET or MRI. Further, tumor stabilization/regression/remission may occur later during the treatment period. Therefore, DeltaRex-G will be continued regardless of CT, PET or MRI results if the patient has clinical benefit and does not have symptomatic disease progression.
Studying Genes in Tissue Samples From Younger and Adolescent Patients With Soft Tissue Sarcomas...
Childhood Alveolar Soft-part SarcomaChildhood Angiosarcoma13 moreThis research trial studies genes in tissue samples from younger and adolescent patients with soft tissue sarcomas. Studying samples of tumor tissue from patients with cancer in the laboratory may help doctors learn more about changes that occur in DNA and identify biomarkers related to cancer. It may also help doctors find better ways to treat cancer
Whole Body Magnetic Resonance Imaging With Diffusion Weighted Imaging : Potential Role in Neurofibromatosis...
Whole Body ImagingMagnetic Resonance Imaging4 moreWhole body MRI will be performed in patients with neurofibromatosis Type 1 PURPOSE 1: To determine the total tumor load (neurofibroma) and to diagnose plexiform neurofibromas or malignant peripheral nerve sheath tumors. All patients will be scanned two years after the baseline whole body MRI to investigate to investigate the changes of total tumor load. PURPOSE 2: added value of diffusion weighted imaging in diagnosis of high-risk neurofibromas PURPOSE 3 : to determine the apparent diffusion coefficient of the malignant nerve sheath tumors and neurofibroma. PURPOSE 4 : correlation between histopathology of the surgically resected neurofibroma/malignant nerve sheath tumors and MRI findings
Tazemetostat Expanded Access Program for Adults With Solid Tumors
Epithelioid Sarcoma (Ex-US Only)Spindle Cell Sarcoma18 morePatients with a diagnosis listed under "conditions" below are eligible to be considered for the EAP. These conditions must be serious or life-threatening at the time of enrollment and appropriate, comparable, or satisfactory alternative treatments must have been tried without clinical success. Patients with conditions not listed under "conditions" below are not eligible for the tazemetostat EAP.