Management of Neurofibromatosis of the Nipple-Areolar Complex

Neurofibromatosis type 1 is an autosomal dominant disease having an incidence of 1 in 3000 individuals. It primarily involves the peripheral nervous system and usually presents with many neurofibromas. On rare occasions, NF1 can affect the breast and manifests as nipple-areolar complex extranipple (pseudopolythelia) like neurofibromas which can be disfiguring and sometimes cause pain and therefore need to be addressed surgically. We present a case of a 31-year-old female, who had multiple pedunculated neurofibromas around the nipple on both breasts for 3 years. These lesions were associated with mild pain and were increasing in size. Surgical excision was done while preserving the nipples bilaterally. NF1 primarily involves the peripheral nervous system and usually presents with a large number of neurofibromas. Several case series of patients with NF1 have been reported, but there are only a few published reports on neurofibromas of the nipple-areolar complexes. These lesions can be painful and cause cosmetic deformity. In our case, these lesions were approached by circumferentially excising the redundant nipple-areolar skin containing the neurofibromas, while isolating the nipple on a central ductal and vascular pedicle. In conclusion, the redundant nipple-areolar skin containing the neurofibromas can simply be approached by circumferential excision while preserving the nipple. This technique is simple, easy to perform, while it allows duct preservation and preserves cosmesis.