Case Report

Bilateral Neurofibromas of the Nipple-Areolar Complex: A Case Report and Approach to Diagnosis

Table 1

Differential diagnosis of common breast spindle cell lesions that may involve the nipple [35]. SMA: smooth muscle actin, ER: estrogen receptor, PR: progesterone receptor, DCIS: ductal carcinoma in situ, and CKs: cytokeratins.

Differential diagnosis of common breast spindle cell lesions
BenignHistological FeaturesImmunohistochemistry

FibromatosisCollagen deposition amongst spindle cells arranged in fascicles infiltrating fatty tissue. Lymphoid aggregates may be seen at the margins of the lesion.Positive: SMA, B-catenin (80% nuclear),
Negative: S100M CKs, p63, desmin,CD34

MyofibroblastomaIrregular, nonencapsulated spindle cells with collagen bundles. Rare/No mitoses. No epithelial component seen.Positive: desmin, SMA, CD34, ER, AR caldesmon, vimentin, Bcl2 and CD99.
Negative: S100, CKs, p63, RB STAT6

NeurofibromaBundles of wavy spindle cells, elongated nuclei and narrow cytoplasmic processes.Positive: Neurofilament, S100

SchwannomaSimilar to Neurofibroma but characteristic Verocay Bodies with nuclear palisading, Antoni A & B areasPositive: Neurofilament, S100

Nodular FasciitisFibroblasts with vesicular nuclei in myxoid stroma. Mitoses frequently detected. Rapidly growing lesion arising from superficial fasciaPositive: SMA, vimentin,
Negative: S100,CD34,desmin, keratin

Malignant

MPNSTTypes: Glandular, Epitheliod, mesenchymal. Spindle cells in dense cellular areas of high mitotic activity. Areas of necrosis, nuclear pleomorphismPositive: Neurofilament, S100 p53
Negative: EMA, keratin

Metaplastic CarcinomaMalignant epithelial and mesenchymal areas. High mitotic figures, necrosis and nuclear pleomorphism. DCIS may be seen.Positive: CKs, p63, vimentin, keratin
Negative: CD34

Malignant PhyllodesLeaf-like morphology, marked stromal cellularity and nuclear pleomorphism.Positive/Negative: CD34
Negative: CKs

MelanomaCan be composed of epitheliod +/spindle cells. Can have large pleomorphic cells.Positive: S100, Melan A,
Negative: CKs

AngiosarcomaHaemorrhagic mass composed of epitheliod or spindle cells displaying vascular differentiationPositive: CD31,CD34, keratin