Whole Body Magnetic Resonance Imaging With Diffusion Weighted Imaging : Potential Role in Neurofibromatosis

Study Overview
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Brief Summary
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Whole 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

Official Title
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Whole Body Magnetic Resonance Imaging With Diffusion Weighted Imaging : Potential Role in Neurofibromatosis

Conditions
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Whole Body Imaging, Magnetic Resonance Imaging, Neurofibromatosis 1, Diffusion Magnetic Resonance Imaging, Peripheral Nerve Sheath Tumors, Malignant

Intervention / Treatment
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* Other: Additional imaging or surgery


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients with neurofibromatosis type 1, between 6 and 50 years old Exclusion Criteria: Patients who are not allowed to be scanned on MRI (contra-indications: pacemaker ed.)

Ages Eligible for Study
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Minimum Age: 6 Years
Maximum Age: 50 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 |
| --- | --- |
| Neurofibromatosis 1<br>All patients diagnosed with neurofibromatosis type 1. GROUP 1:ADDITIONAL IMAGING OR SURGERY There will be patients with high-risk neurofibromas (potential malignant). These patients will underwent additional examinations or surgery (outside this study). Follow-up MRI within 2 years (study MRI ) GROUP 2: No suspicious lesions at MRI. Follow-up within 2 years(Study MRI). | Other: Additional imaging or surgery<br>* No specific intervention is necessary. If a suspicious lesion is diagnosed on MRI, further investigation will be planned (PET-CT or surgery - biopsy)<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Estimation of total tumor load and diagnosis of high-risk neurofibromas | Estimation of the total tumor load of neurofibromas with whole body MRI (head to knee). Diagnosis of high risk neurofibroma in the chest, abdomen, pelvis, and extremities with T2-weighted sequence and diffusion weighted sequence. | 1 month | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| To diagnose high-risk neurofibroma | Some patients with neurofibromatosis have lesions, pre-malignant or malignant neurofibromas. Additional imaging (PET-CT), a biopsy or surgical treatment is necessary in combination with histopathology of the lesion. | 2 months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Whole Body Imaging, Magnetic Resonance Imaging, Neurofibromatosis 1, Diffusion Magnetic Resonance Imaging, Peripheral Nerve Sheath Tumors, Malignant