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: Diagnostic trial to study the effectiveness of FDG-PET and MR perfusion imaging in detecting disease progression and determining response to treatment in patients who have neurofibromatosis 1 and plexiform neurofibroma.

Condition	Treatment or Intervention
neurofibromatosis 1 and 2 (NF1 and NF2)	Drug: fludeoxyglucose F 18  Drug: gadopentetate dimeglumine  Procedure: diagnostic test  Procedure: magnetic resonance imaging  Procedure: radionuclide imaging  Procedure: tomography, emission computed

Further Study Details: 

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 2D and 3D 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 within 2 years.

Ages Eligible for Study:  up to  25 Years,  Genders Eligible for Study:  Both

Criteria

DISEASE CHARACTERISTICS:

• 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
• 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)

Pennsylvania
      Abramson Cancer Center at the University of Pennsylvania, Philadelphia,  Pennsylvania,  19104,  United States; Recruiting
      Children's Hospital of Philadelphia, Philadelphia,  Pennsylvania,  19104,  United States; Recruiting

Study chairs or principal investigators

Michael Fisher, MD,  Study Chair,  Children's Hospital of Philadelphia   

Study ID Numbers:  CDR0000299006; CHP-724; CHP-IRB-2001-8-2543; NCT00060008
Record last reviewed:  December 2004
Last Updated:  January 6, 2005
Record first received:  May 6, 2003
ClinicalTrials.gov Identifier:  NCT00060008
Health Authority: United States: Federal Government
ClinicalTrials.gov processed this record on 2005-04-08