Tetrad presentation of non-syndromic odontogenic keratocyst: An uphill diagnostic and therapeutic challenge

Abstract

Odontogenic keratocyst (OKC), in the last decade sceptically referred to as keratocystic odontogenic tumor (KCOT), is known for its subclinical extensive growth potential and significant rate of recurrences. Odontogenic keratocyst, being the third most common cystic lesion (10–20%) of the maxillofacial region, is often recognized as a sporadic lesion and is well-documented in the literature. Multiple presentation of these cysts over a lifetime is relatively uncommon and is usually seen in conjunction with nevoid basal cell carcinoma syndrome (NBCC), orofacial digital syndrome, Noonan syndrome, Ehlers–Danlos syndrome, Simpson–Golabi–Behmel syndrome, or other syndromes. The ‘two-hit’ hypothesis postulated by Knudson best explains this anomaly, wherein multiple OKCs associated with the syndromes arise as a consequence of the allelic loss in the patched (PTCH) gene, mapped to the long arm of chromosome 9q22.3-q31. A partial expression of the gene may result in multiple OKCs (5%) without any related syndromes. Though concurrent occurrence of non-syndromic multiple OKCs is a rare phenomenon, a handful of cases have been documented over the past few years. Adding to this, we report a case of multiple OKCs occurring synchronously and bilaterally in all 4 quadrants in non-syndromic, otherwise healthy persons, which could indicate a shift in trend.