PARIS — Resection can't be too early or too aggressive when treating neurofibroma in children, reported McKay McKinnon, M.D., at the Fourth International Academy of Cosmetic Dermatology World Congress.
Neurofibroma is a usually benign tumor of the peripheral nerves caused by abnormal proliferation of Schwann cells.
The development of neurofibromas, multiple café au lait spots, and freckling in the armpits or groin area are common symptoms of neurofibromatosis 1 (NF1), which occurs in 1 in 4,000 births.
There is no way to tell which patients will develop a mild case and which patients will have serious complications such as painful or disfiguring tumors.
Tumors may grow back and in greater numbers, and they could be fibrosarcoma, said Dr. McKinnon, a plastic and reconstructive surgeon with the University of Chicago. Early surgery, even in infancy, may be indicated.
“In my experience, a very aggressive surgery can often produce nonrecurrence for many years or forever,” he said. “That's hard to prove, but I think that should be the principle.”
Viable structure may remain after excision of large tumors from the ear. But ocular tumors pose a wide range of problems, particularly if the tumor extends into the extraocular muscles.
If at all possible, surgeons should not sacrifice the important structures of the face or the facial nerve, he said.
A careful and early diagnosis of neurofibroma is important and can help distinguish NF1 from neurofibromatosis 2 (NF2), which is rarer, occurring in 1 in 40,000 births.
NF2 is characterized by multiple tumors on the cranial and spinal nerves and by other lesions of the brain and spinal cord. Its primary characteristic is bilateral tumors of the eighth cranial nerve, resulting in hearing loss beginning in the patient's teens or early 20s.
Because patients with neurofibroma may have recurrence throughout life and/or develop neurosarcoma, they need continued surgical surveillance, Dr. McKinnon said.
The National Institutes of Health is launching a study to investigate whether genetic differences explain differences in disease severity among patients with NF1. Participants must be at least 16 years old. For more information, write to jsloan@mail.nih.gov