Mandibular ameloblastic carcinoma in a young patient.

Ameloblastic carcinoma is a rare cause of jaw tumors, especially in children. This rare, rapidly growing, malignant tumor of odontogenic origin affects predominantly the mandible and maxilla. Hypercellularity, lack of differentiation, high mitotic index, vascular and neural invasion are its main histological features. Local destruction and distant metastases to the lungs, bones, liver and brain are common in ameloblastic carcinoma. Prognosis is poor, due to its low sensitivity to chemo- and radiotherapy. We report the case of an 8-year-old girl with ameloblastic carcinoma of the left mandible and extensive right pleuro-pulmonary and bone marrow metastases. Biopsies made from the mandibular tumor and lung tumor tissue obtained by bronchoscopy, showed the same histological features, that of ameloblastic carcinoma. Diagnostic and treatment challenges are shown in this uncommon pediatric solid tumor of the jaw. Carboplatin and etoposide treatment showed some therapeutic effect upon the primary tumor, but lung infiltrations were not influenced. Palliative treatment was initiated. Early detection of ameloblastic carcinoma, before massive distant metastases develop would be an option for long-term survival, which can be achieved with radical surgery followed by radiotherapy.