Desmoplastic ameloblastoma (including "hybrid" lesion of ameloblastoma). Biological profile based on 100 cases from the literature and own files.

The profile of the desmoplastic ameloblastoma (DA) is presented, based on a survey of 100 cases. DA is a benign, locally invasive variant of the intraosseous, infiltrative ameloblastoma (IA). Radiologically, the DA shows ill-defined borders with a soap bubble appearance. The finding of a mixed radiolucency-radiopacity in 52% of the cases often leads to a preoperative diagnosis of a fibro-osseous lesion. Association with an unerupted tooth is not a typical finding. The DA accounts for 4-13% of all ameloblastomas. The over-all average age is 42.9 years, higher for males (45.9 years) than for females (39.7 years). Thus, the DA occurs at a slightly higher age than the intraosseous ameloblastoma (37.4 years). The male/female ratio is 1:1. The maxilla/mandible ratio is 1/0.9 as opposed to 1/5.4 for the IA. Seven maxillary tumours involved an entire quadrant and 15 maxillary and mandibular tumours crossed the midline of the jaws. Pathogenetically, it seems that the DA is derived from the same sources as the IA, and extra-osseous sources do not seem to play a role. Histologically, the DA reveals the following morphological characteristics: the odontogenic epithelium occurs as irregular, stellate or follicular islands and cords, the center often appearing hypercellular with spindle-shaped or squamatoid cells. Peripheral columnar or cuboidal cells rarely reveal an ameloblast-like appearance. Thus, there are only vague tendencies to mimic a follicular ameloblastoma with acanthomatous features. The most striking feature separating the DA from IA is to be found in the tumour stroma: in DA there is extensive stromal desmoplasia with an abundance of thick collagen fibres that seem to compress the epithelial islands. Nine cases of so-called "hybrid lesion of ameloblastoma" where areas of IA coexist with areas of DA are surveyed in addition to the 100 cases of DA. No conclusions can be drawn about the biological profile of this variant due to the insufficient number of cases.