The intraepithelial deposit of perlecan, a basement membrane-type heparan sulfate (HS) proteoglycan, has been demonstrated in neoplastic conditions such as salivary gland tumors, odontogenic tumors, and oral carcinoma in situ.
A review of the biological behavior of this recognized aggressive pathological entity of the jaws and a contemporary outline of the molecular (growth factors, p53, PCNA and Ki-67, bcl-2) and genetic (PTCH, SHH) alterations associated with this odontogenic neoplasm provides a better understanding of the mechanisms involved in its development and strengthen the current concept that the KCOT should, indeed, be regarded as a neoplasm.
Alterations in the Sonic Hedgehog signaling pathway, including PTCH gene mutations, have been associated with the pathogenesis of some odontogenic tumors.
Stromal cells were isolated from the fibrous capsules of patients with NBCCS-associated or non-syndromic keratocystic odontogenic tumors and non-syndromic tumor stromal cells without PTCH1 mutations served as controls.
These findings further confirmed that CTNNB1 mutation is not frequent in ameloblastoma and malignant odontogenic tumors, although the abnormality of Wnt signaling may be associated with some of these tumors.
Altered expression of cell-cell adhesion molecules β-catenin/E-cadherin and related Wnt-signaling pathway in sporadic and syndromal keratocystic odontogenic tumors.
Sequencing of the all encoding region of AMBN gene was carried out in four frozen cases of odontogenic tumors: one case of calcifying epithelial odontogenic tumor (CEOT), two calcifying odontogenic cysts (COC) and one ameloblastic fibroma (AF).
The higher expression rates of IL-1α and IL-6 were associated with tumor size in ameloblastomas and with cyst wall thickness in keratocystic odontogenic tumors.
Our findings suggest that aberrant beta-catenin expression and APC missense mutation may play an important role for the pathogenesis of epithelial odontogenic tumors.
Our data demonstrates that AMBN gene has an important role in the tumorigenesis of subtypes of epithelial odontogenic tumors and that this phenotypic heterogeneity could be caused by genetic heterogeneity.
Thus, our results suggest that AMBN promotes cell binding through the heparin binding sites and plays an important role in preventing odontogenic tumor development by suppressing cell proliferation and maintaining differentiation phenotype through Msx2, p21, and p27.
This immunohistochemical study using MDM2 expression was undertaken to understand better the diverse biological activity of two groups of odontogenic tumors namely ameloblastoma and adenomatoid odontogenic tumor (AOT) based on their cell proliferation activity.
Although recent identification of BRAFV600E mutation and subsequent activation of mitogen-activated protein kinase (MAPK) pathway in ameloblastoma and odontogenic tumors provide additional options with targeted therapeutics, the molecular background of OKC is not well understood.