A rare case of calcifying epithelial odontogenic tumor (CEOT) devoid o
f calcification is reported with histochemical, immunohistochemical an
d electron microscopic studies. The tumor occurred intraosseously in t
he left maxillary canine and premolar region of a 58-year-old man. The
tumor chiefly consisted of scattered small islands of epithelial cell
s in an abundant fibro-myxoid connective tissue stroma. Among the nest
s, there were many spherical bodies of eosinophilic substance for whic
h non-AA amyloid and non-keratin or basal lamina-like natures were dem
onstrated histochemically and immunohistochemically. In some nests, th
ere were a few, occasionally several, cells positive for S-100 protein
. Ultrastructurally, Langerhans cells with indented nuclei and Birbeck
's granules were seen among tumor cells. The prognostic significance o
f the paucity of calcification and the presence of Langerhans cells in
CEOT of which this is only the second description is discussed.