If yes, is it coincidental or a correlation? Gardner's syndrome is known to be associated with intestinal polyposis, supernumerary tooth, impacted tooth, odontomas, osteomas.
Since, the syndrome is associated with supernumerary tooth/impacted tooth, can the odontogenic keratocyst occur in these cases?
The clue probably would be the PTCH gene. Supernumerary teeth may involve the Wnt, Notch, SHH etc while these genes could be influenced by PTCH mutation - exon7 see - Int J Mol Med. 2006 May;17(5):755-9.
This is just a hypothesis and probability
All the best
Yes.In Gardner syndrome occur OKC with high recurrence after removal (nearly %60)
and okc is susceptible to develop basal sell carcinoma.
Hello,
I disagree with both Dr Thavarajah and Dr Ghaderi (although their comments can be slightly mistaken since two syndromes have similar names) and here´s why:
The PTCH is mutated in Gorlin´s syndrome, multiple basal cell carcinoma- and developmental malformation cases, but not in Gardner´s syndrome (http://www.ncbi.nlm.nih.gov/gene/5727).
This is also stated in the manuscript which Dr Thavarajah refers to ( Int J Mol Med. 2006 May;17(5):755-9 http://www.spandidos-publications.com/ijmm/17/5/755 ).
In Gorlin´s syndrome, PTCH is associated with KCOTs (keratocystic odontogenic tumours) and the SHH-PTCH pathway is likely altered. However, in Gardner´s with supernumerary teeth this is not the case. I gave Dr.Thavarajah´s theory a long thought and find it unlikely to be probable: In Gardner´s there are supernumeraries in most of patients, (http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3258555/#B5) and in supernumerary toothbuds the Wnt pathway is altered and this could be activated via the shh-PTCH pathway as stated by Musani et al. in the manuscript Dr.Thavarajah refers to. However, two points lead me to believe this would not lead to KCOT occurrence:
1) No cases of KCOT have been reported in Gardner´s (to my knowledge) and
2) the critical PTCH mutation is not present to cause KCOT because PTCH resides upstream of Wnt. Musani et al. state that PTCH could activate Wnt but not the other way around (since it is upstream) and therefore could not affect KCOT pathogenesis. This is just my speculation about the matter, please do correct me if you have more information!
In Gardner´s syndrome there is a prevalent chromosome 5 (5q21-22) mutation, also known as the FAP gene (see Int J Med Sci. 2012 http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3258555/).
This has been reported to lead to, in addition to the symptoms mentioned by Dr. Babu in OP, to increased dentigerous cyst formation.
In Imaging Sci Dent. 2012, Mali et al. describe supernumerary teeth in Gardner´s (http://www.ncbi.nlm.nih.gov/pubmed/22474647 ). They report that, as supernumerary teeth more often develop pathologies (cysts), an increased dentigerous cyst formation probably is present. This is the only article I found in the literature mentioning actual dental cysts in Gardner´s. No direct mention of KCOTs are found in their work, either.
In Gardner´s syndrome, there is a multitude of epidermoid cysts that somewhat resemble histologically KCOTs but are from the skin and as such, entirely separate entities. Also, as I already stated, there are no reported cases of KCOTs in Gardner´s syndrome and it must be concluded that if ever someone diagnosed such a cyst in a Gardner´s patient, it would probably be a coincidental finding or something worthy of reporting.
I also disagree with Dr. Ghaderi.
There are no reports of OKCs having been found due to Gardner´s-, but there are several due to Gorlin´s syndrome.
Also, no transformation into basal cell carcinoma has been reported in KCOTs. Squamous cell carcinomas have been reported, however, but are luckily very rare.
Please provide references to your claims. If I haven´t found the literature in my search, I have been mistaken and would gladly be corrected.
Finally, to answer Dr. Babu´s original question: I think that if there is an increased incidence in KCOTs in Gardner´s syndrome, it reflects the increased incidence of supernumerary teeth that are associated with the condition. However, the literature does not (yet) support this. For some reason, the pathways that are altered in Gorlin´s sdr (PCHT) lead to KCOT and also supernumerary teeth formation (http://www.ncbi.nlm.nih.gov/pubmed/22669069) but the pathways altered in Gardner´s (FAP) only lead to supernumerary teeth but not KCOTs.
The old cliché remains; more studies are needed.
Best regards,
Aaro Turunen
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