Multi-parametric Biomarker Development to Predict Malignant Conversion in Patients With Neurofibromatosis Type 1

The 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.

Inclusion Criteria:Inclusion criteria are person (age > 6 years) with clinical diagnosis of NF1, ability to undergo whole body MRI without intravenous contrast material considered "high-risk" for MPNST.The term "high risk" is defined as:high pNF burden defined as 1 pNF > 3 cm or > 1 pNFdiagnosis of whole-gene deletion ("microdeletion") of the NF1 gene on genetic testingprior history of atypical or malignant PNSTfamily or personal history of MPNST or atypical PNSTprior radiation treatmentExclusion Criteria:Pregnancyinability to tolerate MRI or 18F-FDG-PET/CT imaging without anesthesiaongoing NF1-related intervention (including systemic steroids) or therapy that may alter the anatomic, metabolic or functional MRI appearance of the PNSTs.