Management of Neurofibromatosis of the Nipple-Areolar Complex

Abstract

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.

Neurofibroma of breast- A rare case report

“…This case with unilateral nipple areola complex neurofibroma presenting in a female displays an unusual presentation and diagnosis within histopathology with a vast array of differential diagnosis. 8 Immunohistochemistry plays a crucial role in the diagnosis of breast spindle cell lesions. 8 Solitary neurofibroma of breast is an uncommon benign tumour which can mimic other more aggressive tumours.…”

“…8 Immunohistochemistry plays a crucial role in the diagnosis of breast spindle cell lesions. 8 Solitary neurofibroma of breast is an uncommon benign tumour which can mimic other more aggressive tumours. Imaging plays an important role, however, diagnosis relies on pathology.…”

“…Imaging plays an important role, however, diagnosis relies on pathology. 8 MRI is the best imaging technique to diagnose morphology and enhancement pattern suggestive of a benign lesion. 9…”

A Case Report: Massive Spontaneous Hemorrhage of Neurofibromatosis Type 1 on Upper Back

“…Definite treatment to prevent re-bleeding is considered to be surgical operation including total/subtotal resection and vessel ligation in literatures [1,5,6].…”

“…It 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 [1]. Cutaneous neurofibromas are hallmarks of Neurofibromatosis type 1 (NF1) and cause the main disease burden in adults with NF1 [2].…”