Odontogenic keratocyst: imaging features of a benign lesion with an aggressive behaviour

The latest (4th) edition of the World Health Organization (WHO) Classification of Head and Neck Tumours, published in January 2017, has reclassified keratocystic odontogenic tumour as odontogenic keratocyst. Therefore, odontogenic keratocysts (OKCs) are now considered benign cysts of odontogenic origin that account for about 10% of all odontogenic cysts. OKCs arise from the dental lamina and are characterised by a cystic space containing desquamated keratin with a uniform lining of parakeratinised squamous epithelium. The reported age distribution of OKCs is considerably wide, with a peak of incidence in the third decade of life and a slight male predominance. OKCs originate in tooth-bearing regions and the mandible is more often affected than the maxilla. In the mandible, the most common location is the posterior sextant, the angle or the ramus. Conversely, the anterior sextant and the third molar region are the most common sites of origin in the maxilla. OKCs are characterised by an aggressive behaviour with a relatively high recurrence rate, particularly when OKCs are associated with syndromes. Multiple OKCs are typically associated with the nevoid basal cell carcinoma syndrome (NBCCS), an autosomal dominant multisystemic disease. Radiological imaging, mainly computed tomography (CT) and, in selected cases, magnetic resonance imaging (MRI), plays an important role in the diagnosis and management of OKCs. Therefore, the main purpose of this pictorial review is to present the imaging appearance of OKCs underlining the specific findings of different imaging modalities and to provide key radiologic features helping the differential diagnoses from other cystic and neoplastic lesions of odontogenic origin.