Pediatric odontogenic keratocyst and early diagnosis of Gorlin syndrome: Clinicopathological aids.

Odontogenic keratocysts (OKCs) are a common presentation in almost all patients with nevoid basal cell carcinoma syndrome (NBCCS) or Gorlin syndrome, irrespective of race. In most patients with NBCCS, OKC presents as multiple lesions affecting the jaws which makes it a signpost for the investigation of patients with the syndrome. In approximately 40% of pediatric patients, the initial presentation is that of a single OKC, which may often result in missing the diagnosis of NBCCS. This is particularly common in patients without clinically apparent NBCCS-related manifestations. This review examines the clinicopathological features that clinicians and oral pathologists may look for in pediatric patient with OKC and OKC surgical specimens that may serve as indicators for the diagnosis of NBCCS. Although these features do not diagnose NBCCS by themselves, they may significantly help in initiating the diagnostic process at an early stage with an obvious benefit to the child and relatives.