Genetic alterations in syndromes with oral manifestations
A female patient aged 10 years, reported to our college with the complaint of halitosis and decayed tooth. Clinical examination revealed no obvious changes []. An orthopantomograph revealed unilocular radiolucencies with scalloped margins in all the four quadrants of maxilla and mandible []. Biopsy was carried out and histopathology showed a cystic lining comprised of parakeratinized epithelium with palisading basal layer along with connective tissue wall [], suggestive of odontogenic keratocyst (OKC).\nA diagnosis of multiple OKC was arrived at, based on these findings. However, she did not have any other manifestations relating to a Nevoid Basal cell Carcinoma Syndrome (NBCCS). Chest radiograph did not show any relevant features []. Gene mutation leading to sporadic OKC was mapped to the Patched (PTCH) gene, a tumor suppressor gene at 9q22.3.