Natural History of Plexiform in Neurofibromatosis Type I

Inclusion Criteria

- INCLUSION CRITERIA:

Subjects must fulfill two or more of the following diagnostic criteria for NF1:

Six or more cafe-au-lait macules (1. 1.5 cm or larger in postpubertal individuals; 2. 0.5
cm or larger in prepubertal individuals);

Two or more neurofibromas of any type or 1 or more plexiform neurofibroma;

Freckling in the axilla or groin;

Optic glioma (tumor of the optic pathway);

Two or more Lisch nodules (benign iris hamartomas);

A distinctive bony lesion (1. Dysplasia of the sphenoid bone; 2. Dysplasia or thinning of
long bone cortex);

A first degree relative with NF-1.

EXCLUSION CRITERIA:

Presence of metallic implant that will make the patient unable to have MRI studies.

Presence of a medical or psychological condition that will make the patient unable to
tolerate MRI studies or anesthesia (if needed).

Inability to image tumor or define tumor margins by MRI (which may be determined after the
initial study).

Failure to obtain initial MRI within 60 days of enrollment.

Previous radiation therapy to site of plexiform neurofibroma.

Surgery involving the plexiform neurofibroma (excluding biopsy) within a six month period
before enrollment.

Current antineoplastic therapy.

Entry of more than one member of the same family into the study is not permitted.