Calcifying odontogenic cyst: Report of an uncommon entity with a brief literature review.

The aim of this clinical report is to document a rare and unusual case of calcifying odontogenic cyst (COC) in the maxillary anterior region in a 13-year-old girl. A COC is an extremely uncommon developmental, odontogenic entity and accounts for 0.3%-0.8% of odontogenic cysts. The lesion presents as an array of varied radiographic and clinicopathological characteristics and biological attributes and exists in three histomorphologic patterns - benign cystic, solid (neoplastic) and aggressive (malignant) forms. Thus, several nomenclatures and classifications have been put forth to explain the nature of the clinical entity. However, ambiguities regarding the exact nature of the lesion still prevail. Due to nonspecific clinicoradiographic features, histopathological interpretation remains the key for diagnosis. We report an uncommon occurrence of COC in a 13-year-old female who reported to our Outpatient Department with an asymptomatic right midfacial swelling. The clinical and radiographic findings were suggestive of adenomatoid odontogenic tumor and dentigerous cyst. The decision to enucleate the lesion was considered, and histopathological features were compatible with the diagnosis of COC. Re-ossification with no recurrence was noticed after a 1-year follow-up. COC is an unusual developmental odontogenic cyst that clinically and radiologically simulates other more common jaw entities. Thorough knowledge of the bizarre presentation and biological attributes of such lesions are imperative for an early diagnosis and definitive treatment. Long-term follow-up is advocated to prevent recurrences.