Primary squamous cell carcinoma of the breast is a rare clinical entity. Tw
o large review series found only five cases out of a total of 8351 breast m
alignancies. This case report presents a patient with metaplastic, pseudoan
giosarcomatous carcinoma or acantholytic variant of a squamous cell carcino
ma of the breast. This diagnosis was based on the histological finding of h
ighly atypical, acantholytic squamous cells. Because the tumor stained posi
tive for keratin and negative for factor VIII, the diagnosis of angiosarcom
a was ruled out. Although only scattered case reports have been published o
n this histological variant, these tumors tend to follow an aggressive cour
se.