Background. Ameloblastic carcinoma is a rare, aggressive odontogenic n
eoplasm of the jaws in which the epithelial cells exhibit cytologic fe
atures of recognizable ameloblastoma and malignancy. Cases with metast
asis have been infrequently reported. Methods. A case of a 64-year-old
white woman with mandibular ameloblastic carcinoma with documented di
stant metastasis is presented. The patient's presenting symptoms inclu
ded facial asymmetry of the right jaw over 2 months and the developmen
t of moderate trismus. Clinical manifestations, pathology, treatment,
and biologic behavior are discussed, The nomenclature and classificati
on of odontogenic carcinomas are reviewed, including entities that sho
uld be considered in the differential diagnosis. Results. The patient
underwent surgical resection consisting of mandibulectomy, parotidecto
my, and modified radical neck dissection followed by radiation to both
necks and tumor bed, Postsurgically, the patient developed pulmonary
metastasis at 11 months and expired with widespread metastatic disease
at 28 months. Conclusions. This case demonstrated an unusual behavior
pattern in that local recurrence and regional metastasis did not occu
r, Distant metastasis occurred despite apparent adequate control of th
e primary mandibular tumor. The ameloblastic carcinoma is a highly mal
ignant neoplasm which requires aggressive therapy. Prognosis is poor.
Further reporting of ameloblastic carcinoma is encouraged. (C) 1998 Jo
hn Wiley & Sons, Inc.