A Case of multiple mandibular and maxillary cysts, could it be Gorlin-Goltz syndrome?

by April 9, 2021
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A Case of multiple mandibular and maxillary cysts, could it be Gorlin-Goltz syndrome?

Uma S, Shim CK, Lisamarie S: A Case of multiple mandibular and maxillary cysts, could it be Gorlin-Goltz syndrome? Mal. J. Oral Maxillofac. Surg. 2020; 18: 20–26.

Abstract: The odontogenic keratocyst (OKC) is known for its high recurrence rate, aggressive behavior, and its occasional association with the nevoid basal cell carcinoma syndrome (NBCCS). NBCCS, also referred to as Gorlin-Goltz syndrome, is associated with a gene level disturbance of chromosome 9 and is transmitted as an autosomal dominant trait with high penetrance. Multiple odontogenic keratocysts are a well- recognized feature of NBCCS. Clinically, OKC occurs over a wide age range with a peak in the second and third decades. It has a marked male predilection. OKCs associated with the syndrome tend to be more aggressive and have higher recurrence rates compared with solitary keratocysts. This article presents a case of multiple OKCs in the maxilla and mandible and other cardinal features manifested in Gorlin Goltz syndrome patients. Early recognition and diagnosis of the disease with detailed family history is essential for the management of this syndrome.