fludeoxyglucose f 18 positron emission tomography and magnetic resonance perfusion imaging in patients with neurofibromatosis 1 and plexiform neurofibroma

Conditions: Neurofibromatosis Type 1; Precancerous Condition

Summary: 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.

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.

Eligibility criteria:
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)

Keywords: neurofibromatosis type 1 plexiform neurofibroma