Watchful Waiting Best in Kids' Neurofibromatosis

NF1 patients with neurofibromas have a 10% lifetime risk of developing a malignant peripheral nerve sheath tumor within one of the lesions. Signs of malignant degeneration include persistent pain, a change in texture, a rapid increase in size, or development of a neurologic deficit associated with the neurofibroma.

Dr. Bird said she had no relevant disclosures to make.

Cafe au lait macules, shown here, are usually the first sign of NF1.

Axillary freckling is often evident later in the school-age child.

The Riccardi sign, a tuft of hair near the spine, may be present at birth and may even precede macules. Photos courtesy Dr. Lynne M. Bird and Dr. Marilyn C. Jones