Case report on multiple keratocystic odontogenic tumors of jaws: Comparison of a non-syndromic case versus a case of Gorlin Goltz Syndrome

Publication date: October 2014 Source:Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology, Volume 26, Issue 4 Author(s): Priya Jeyaraj , Nitesh Naresh , V. Srinivas The presence of multiple Keratocystic odontogenic tumors (KCOTs) of the jaws in a patient must alert a clinician as to the possibility of a syndromic condition. The Gorlin Goltz syndrome (GGS), otherwise known as the Nevoid basal cell carcinoma (NBCC) syndrome, and as the Jaw cyst-Basal cell Naevus-Bifid rib syndrome is one such possibility, which is typically characterized by a classical triad as established by Gorlin and Goltz in 1960, comprising of multiple basocellular epitheliomas, keratocysts in the jaws and bifid ribs. A spectrum of other neurological, ophthalmic, endocrine and genital manifestations is known to be variably associated with this triad. Two cases of multiple KCOTs of the jaws have been presented, one syndromic, while the other, non-syndromic. A comparison of the essential and distinguishing features of both, along with the importance of a thorough medical evaluation and correct diagnosis for appropriate treatment have been highlighted. These investigations prompt an early verification of the disease, which is very important to prevent recurrence and better survival rates from the co-existent diseases.
Source: Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology - Category: ENT & OMF Source Type: research