Histology: Individual neurofibromas have a well-circumscribed, spindle-shaped proliferation within the dermis. No capsule is present. Schwann cell proliferation and proliferation of the axonal components of the nerve are seen. Many mast cells are present in these tumors. The epidermis is uninvolved, and a small grenz zone is often appreciated.
Treatment: Definitive treatment of a solitary neurofibroma is complete excision. This is curative and results in a very low recurrence rate. No treatment is necessary, because the transformation into malignancy is extremely low.
Any neurofibroma that starts growing or becomes hard or tender should be removed to look for degeneration into neurofibrosarcoma.
Patients with neurofibromatosis require a multidisciplinary approach and need to see a good internist to coordinate all the potential systemic complications. The neurofibromas may be removed surgically. This approach is not ideal, because the number of lesions typically precludes removal of only the bothersome ones. Plexiform neurofibromas should be removed by a plastic surgeon, because they can have large subcutaneous extensions that are not visible clinically. There is no cure for this genetic disease; lifelong screening and follow-up are required, and the patient should be referred for genetic counseling before reaching child-bearing age.
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