The lesion traditionally known as odontogenic keratocyst has been renamed by WHO in 2005, as "keratocystic odontogenic tumor" as it is more appropriate and reflects its potential for local, destructive behavior. It is a benign intraosseous neoplasm of jaw, which is unusual due to its characteristic histopathological and clinical features, including potentially aggressive behavior, high recurrence rate and association with the nevoid basal cell carcinoma syndrome. The purpose of this review is to highlight the importance of proper diagnosis of keratocystic odontogenic tumor in order to prevent the recurrence due to improper surgical excision of the lesion.

Gorlin-Goltz syndrome is an autosomal dominant disorder with a high degree of penetrance. It is characterized by basal cell carcinomas, odontogenic keratocysts, palmar and plantar pits and ectopic calcifications of falx cerebri. The presence of two major and one minor criteria or one major and three minor criteria are necessary to establish a diagnosis. Early diagnosis and treatment of Gorlin-Goltz syndrome, as well as family screening and genetic counseling are essential as it may be associated in 10% of patients with aggressive basal cell carcinoma and malignant neoplasias. We report here a patient with Gorlin-Goltz syndrome.

The Adenomatoid Odontogenic Tumor (AOT) is a rare, slow growing, benign, odontogenic epithelial tumor with characteristic clinical and histological features; which usually arise in the second or third decade. It is a tumor composed of odontogenic epithelium in a variety of histoarchitectural patterns which are embedded in a mature connective tissue stroma. It is mostly encountered in young patients with a greater predilection for females. Maxilla is the predilection site of occurrence, most commonly associated with an unerupted maxillary canine. It presents as a symptom-free lesion and is frequently discovered during routine radiographic examination. This case report describes an unusual case of 20 year old male with only a one month history of tumor in the anterior mandible. The tumor was a well circumscribed intraosseous lesion with an embedded tooth. Histological evidence of calcification was present. The present case lends support to the categorization of AOT as a mixed odontogenic tumor.