PET/MRI in CNS and Extra-CNS Tumors of Patients With Neurofibromatosis-1 (NF1)

Study Overview
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Brief Summary
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This prospective pilot study is designed to provide preliminary data on the use of Fluorodeoxyglucose Positron Emission Tomography-Magnetic Resonance Imaging (FDG-PET-MRI) in patients with neurofibromatosis-1 (NF1) associated optic glioma and plexiform neurofibroma (PN). Subjects will undergo FDG-PET-MRI scans in place of standard of care imaging at 0 and 12 months, unless more frequent imaging is clinically indicated. Subjects and their family caregivers will also undergo serial interviews and complete questionnaires related to the psychosocial aspects of NF1.

Official Title
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Pilot Study of [18F] Fluorodeoxyglucose Positron Emission Tomography- Magnetic Resonance Imaging (FDG-PET-MRI) in CNS and Extra-CNS Tumors of Patients With Neurofibromatosis-1 (NF1)

Conditions
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Neurofibromatosis-1, Optic Glioma, Plexiform Neurofibroma

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Diagnosed with neurofibromatosis-1 with either optic glioma, due for imaging scan, or plexiform neurofibroma, due for imaging secondary to clinical signs or symptoms of progressive disease ≥ 6 years of age English-speaking If female of child-bearing potential, negative urine pregnancy test performed within 7 days prior to each FDG-PET-MRI Study-specific informed consent or assent obtained and signed Exclusion Criteria: Unable to undergo FDG-PET-MRI without sedation Currently undergoing chemotherapy for progressing optic glioma Pregnant or lactating female Poorly controlled diabetes mellitus Presence of pacemaker, intracranial aneurysm clip, cochlear implant, metal halo device, epicardial pacemaker leads, or any other device that makes MRI unsafe Serum creatinine > 1.8 mg/dL OR GFR < 30 mL/min Unable to lie flat for > 1 hour Body Mass Index (BMI) > 35

Ages Eligible for Study
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Minimum Age: 6 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Plexiform Neurofibroma<br>NF1 associated plexiform neurofibroma | |
| Optic Glioma<br>NF1 associated optic glioma | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Plexiform Neurofibroma Cohort: Difference in FDG-avidity between progressive and non-progressive lesions | Ratios of SUVmax values within a group of patients who all have progressive disease | 12 months | 
| Optic Glioma Patients: Compare FDG-avidity between patients with progressive and non-progressive disease |  | 12 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Report descriptive statistics for FDG-PET-MRI results | SUV max, tumor/brain uptake ratio, tumor/muscle uptake ratio | 0, 12 months | 
| Explore impact of FDG-PET-MRI surveillance on patient and family caregiver uncertainty and psychological distress |  | 0, 12 months |