Imaging appearance of Isolated diffuse neurofibroma of nipple areolar area: a case report

Abstract

Sporadic neurofibromas of the nipple-areolar complexes are exceptional even in patients with neurofibromatosis. Diffuse neurofibroma is an uncommon subtype of neurofibroma that has received little attention in the imaging literature. As are most superficial lesions, it is often evaluated clinically and if biopsy is needed, it is usually performed without imaging. However the imaging data is quite characteristic with the aim of evaluating the extension in depth and detecting an underlying cancer. We report a case of women without a history of neurofibromatosis presenting a skin thickening disfiguring her left breast, related to diffuse neurofibroma of the nipple-areolar complexes confirmed histologically. We study echo-mammography and breast magnetic resonance imaging (MRI) findings in order to highlight its radiographics features.

Mesenchymal Tumors of the Breast: Fibroblastic/Myofibroblastic Lesions and Other Lesions

“…Localized: Localized neurofibromas account for 90% of these lesions [78,79,81]. Clinically, they are lobular skin-colored lesions with pathognomonic "button-hole sign [81]".…”

“…Sonographically, localized lesions appear as superficial, circumscribed nodules that are hypoechoic and resemble cysts [79] or fibroadenomas [78]. Diffuse lesions can appear hyperechoic, containing multiple interconnected hypoechoic tubular structures and lesion extent is often difficult to determine [81]. Mammographically, they are hyperdense, wellcircumscribed lesions that can be missed given their propensity to occur posteriorly (retroglandular fat or pectoralis fascia) [78].…”