Fludeoxyglucose F 18 Positron Emission Tomography and Magnetic Resonance Perfusion Imaging in Patients With Neurofibromatosis 1 and Plexiform Neurofibroma

Study Overview
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Brief Summary
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RATIONALE: New imaging procedures such as fludeoxyglucose F 18 positron emission tomography (FDG-PET) and magnetic resonance (MR) perfusion imaging may improve the ability to detect disease progression, help doctors predict a patient's response to treatment, and help plan the most effective treatment. PURPOSE: This diagnostic trial is studying how well FDG-PET and MR perfusion imaging work in finding disease progression and determining response to treatment in patients with neurofibromatosis 1 and plexiform neurofibroma.

Detailed Description
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OBJECTIVES: Determine whether fludeoxyglucose F 18 positron emission tomography (FDG-PET) and MR perfusion studies can predict plexiform neurofibroma growth rates in patients with neurofibromatosis 1. Determine whether FDG-PET and MR perfusion studies can predict the likelihood of response in patients who are undergoing investigational treatment for plexiform neurofibromas. Identify neuroimaging characteristics that distinguish patients who have responded to therapy from those who have not after completion of treatment. OUTLINE: Stratum 1: Patients undergo MR perfusion scan with gadopentetate dimeglumine and fludeoxyglucose F 18 positron emission tomography (FDG-PET) at baseline and quantitative MRI evaluation at baseline and 1 year. Stratum 2: Patients undergo quantitative MRI, MR perfusion scan with gadopentetate dimeglumine, and FDG-PET at baseline and 1 year. PROJECTED ACCRUAL: A total of 48 patients (32 for stratum 1 and 16 for stratum 2) will be accrued for this study.

Official Title
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Novel Imaging Modalities For Plexiform Neurofibromas

Conditions
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Neurofibromatosis Type 1, Precancerous Condition

Intervention / Treatment
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* Radiation: fludeoxyglucose F 18
* Radiation: gadopentetate dimeglumine


Participation Criteria
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Eligibility Criteria
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DISEASE CHARACTERISTICS: Stratum 1: Diagnosis of neurofibromatosis 1 (NF1) and plexiform neurofibromas At high risk for progression, as defined by any of the following: Anatomic location such that progression carries a high risk of impairment of function, pain, or disfigurement (e.g., neck/mediastinum, paraspinal nerve roots, orbit, and face) Tumors that the patient, family, or caregiver believes have increased in size within the past year, but appear stable by standard clinical or radiographic measures No plexiform neurofibromas that are small, cause no pain or functional impairment, or are not likely to cause pain or functional impairment over the succeeding 12 months Stratum 2: Diagnosis of NF1 and progressive plexiform neurofibromas Neurofibroma progression documented by increase in lesion size on MRI Currently being enrolled on a clinical therapeutic trial at Children's Hospital of Philadelphia PATIENT CHARACTERISTICS: Age 25 and under Performance status Not specified Life expectancy Not specified Hematopoietic Not specified Hepatic Not specified Renal Not specified Other Not pregnant or nursing Negative pregnancy test PRIOR CONCURRENT THERAPY: Biologic therapy Not specified Chemotherapy Stratum 1: No prior or concurrent chemotherapy No concurrent enrollment on a chemotherapy clinical trial Stratum 2: At least 4 weeks since prior chemotherapy Endocrine therapy Not specified Radiotherapy At least 6 weeks since prior radiotherapy (stratum 2) Surgery Prior surgery for progressive plexiform neurofibroma allowed if incompletely resected and measurable disease remains (stratum 2)

Ages Eligible for Study
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Maximum Age: 25 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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Design Details

Primary Purpose: Diagnostic
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: 18FDG-PET scan and MR perfusion<br>Subjects will undergo MRI for quantitative (2D and 3D) evaluation of plexiform neurofibroma size, MR perfusion scan, and fludeoxyglucose (18FDG) PET scan at the time of study entry. Subjects who are treated for plexiform neurofibroma will undergo another 18FDG PET scan after one year of study entry. | Radiation: fludeoxyglucose F 18<br> <br> Radiation: gadopentetate dimeglumine<br> <br> |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Tumor Progression as Measured by Tumor Area and Volume at 1 Year. | We correlated SUVmax and change in tumor volume over the subsequent year | One year | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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neurofibromatosis type 1, plexiform neurofibroma