Appendices of the nipple and areola of the breast in Neurofibromatosis type 1 patients are neurofibromas.

In NF1-patients, tumours arising in the areola region of the breast often appear similar to the nipple. The aim of this study was to analyse tumours of this region. Eighty-three specimens from 55 patients with NF1 were analysed [females: 42, males: 13, mean age of the total group: 41.6 years, range: 4-62 years]. NF1 patients with apparent extensive affections of the breast who received debulking procedures for disfiguring invasive or superficial plexiform neurofibroma were excluded. All specimens were found to be composed entirely from neurofibromas with no residues resembling segments of glandular structures. Following ablative surgery, no local recurrence was noted within a follow-up period of 10 years. Tumours arising in the areola and nipple area and mimicking an accessory nipple were all neurofibromas. Local excision of neurofibromas was adequate to relief patients from an often unsightly appearance.