An ameloblastic fibrosarcoma (AFS) arising in the maxilla of a 14-year
-old male is described. The tumor originated from the alveolar bone of
the right maxilla with no apparent history of pre-existing lesion, Hi
stologically, the lesion was composed of benign-appearing epithelial i
slands and strands scattered within an exceedingly cellular mass of me
senchymal tissue comprising a large number of stellate- and spindle-sh
aped fibroblast-like cells with marked pleomorphism. Occasional cement
um-like calcification was also noted. Immunohistochemically, the neopl
astic mesenchymal cells were positive only for vimentin, whereas the a
meloblast-like epithelial component showed a distinctly positive react
ion for wide-spectrum keratin and squamous cytokeratin. Clinicopatholo
gical features of the current case, as well as previously reported exa
mples of AFS originating from the maxilla, are briefly discussed.