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From the Pediatric Oncology Branch (E.D., A.J.G., E.F., F.M.B., B.C.W.), NCI, Bethesda, MD; Medical Numerics, Inc. (J.S.), Sterling, VA; Diagnostic Radiology Department (N.P.), National Institutes of Health, Clinical Center, Bethesda, MD; University of Alabama at Birmingham (B.R.K.), Birmingham, AL; Mayo Clinic (D.B.-V.), Rochester, MN; Children’s National Medical Center (R.J.P.), Washington, DC; Children’s Hospital of Philadelphia (J.B.), Philadelphia, PA; Children’s Memorial Hospital (S.G.), Chicago, IL; Children’s Hospital of Pittsburgh (R.J.), Pittsburgh, PA; Dana Farber Cancer Institute (M.K.), Boston, MA; and Biostatistics and Data Management Section (S.M.S.), CCR, National Cancer Institute, Bethesda, MD.
* To whom correspondence should be addressed. E-mail: dombie{at}mail.nih.gov.
Abstract-- Objective: To longitudinally analyze changes in plexiform neurofibroma (PN) volume in relation to age and body growth in children and young adults with neurofibromatosis type 1 and inoperable, symptomatic, or progressive PNs, using a sensitive, automated method of volumetric MRI analysis. Methods: We included patients 25 years of age and younger with PNs entered in a natural history study or in treatment trials who had volumetric MRI over 
16 months. Results: We studied 49 patients (median age 8.3 years) with 61 PNs and a median evaluation period of 34 months (range 18 to 70). The PN growth rates varied among patients, but were constant within patients. Thirty-four patients (69%) experienced 20% increase in PN volume during the observation period. PN volume increased more rapidly than body weight over time (p = 0.026). Younger patients had the most rapid PN growth rate. Conclusions: Volume increase of plexiform neurofibromas is a realistic and meaningful trial endpoint. In most patients plexiform neurofibroma growth rate exceeded body growth rate. The youngest patients had the fastest plexiform neurofibroma growth rate, and clinical drug development should be directed toward this population. Age stratification for clinical trials for plexiform neurofibromas should be considered.
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