Biology of metastasizing ameloblastoma

The present report of a malignant metastasizing ameloblastoma and a critical review of literature was undertaken in an attempt to better understand the biological potential and behavior of this rare tumor and thus to facilitate its clinical management. Most of the 26 patients with a proven malignant ameloblastoma including the present case had developed multiple recurrences. The lung was the most frequent metastatic site (88%) followed by regional lymph nodes (27%). Furthermore metastases were observed in some cases in the bone, brain, kidney, small intestine and liver. The interval between diagnosis of tumor and manifestation of metastases was long with a median of 11.1 years. The average survival time was 13.1 years. By contrast, the interval between diagnosis of metastatic disease and death was relatively short (median: 2.6 years). The histologic and cytologic pattern of malignant ameloblastoma and of its metastases was not significantly different from that of non-metastatic ameloblastoma. Because of the lack of morphological criteria of malignancy the biological behavior of ameloblastomas cannot be predicted. It is difficult to be certain which factors are important in the delayed induction of metastases. It is suspected that ameloblastomas possess an inherent low grade malignancy which is stimulated by multiple recurrences. It is further assumed that the metastatic tumor cells have a slow growth rate resulting in late clinical manifestation of metastases. When lung metastases occur we recommend their surgical removal in order to prolong live expectancy or even to obtain a curative effect.