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Volume 5 Issue 6 (June, 2017)

Case Reports

MULTIPLE KERATOCYSTIC ODONTOGENIC TUMORS- A PRESENTATION OF GORLIN GOLTZ SYNDROME
Selven Thirumalai, Sumir Gandhi, Inderjot Singh, Navpreet Bedi

Background :Gorlin Goltz syndrome is a rare inherited disorder of multiple Keratocystic odontogenic tumors (KCOT) with cutaneous, dento-osseous, ophthalmic, neurological abnormalities. Characteristic findings include multiple KCOTs and basal cell nevi. The KCOT should be treated aggressively due to its recurrence and complications. Method :We discuss two cases of multiple KCOT in young patients, who presented with primary complaints related to the presence of keratocystic odontogenic tumor and were diagnosed as Gorlin Goltz syndrome based on the presence of major and minor diagnostic criteria. Results :Both our cases presented with complaints related to the presence of KCOTs which were treated surgically without evidence of recurrence till date. Conclusions :In Gorlin Goltz syndrome, KCOT’s are often the first clinical feature to be recognised and could be the only condition for which the patient may seek treatment. It is therefore important that OMF surgeons have knowledge of the main features of this syndrome and a high index of suspicion be present when a patient of multiple KCOT presents to our clinics.
Keywords : Keratocystic odontogenic tumor, KCOT, Gorlin Goltz Syndrome,  Odontogenic keratocyst, NBCCS.

 
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