Recurrent Odontogenic Keratocysts of the Mandible in Association with Gorlin–Goltz (Nevoid Basal-Cell Carcinoma) Syndrome: A Case Report

Abstract

Objective: Odontogenic keratocysts (OKCs) are relatively rare benign intraosseous lesions of odontogenic origin. In 2005, the World Health Organization (WHO) reclassified OKCs as keratocystic odontogenic tumors due to their locally aggressive and recurrent behavior. However, the “tumor” designation was later reconsidered. In the 2017 WHO classification the term “odontogenic keratocyst” was reinstated. OKCs are typically solitary lesions presenting radiographically as unilocular or multilocular radiolucencies, most often in the mandibular molar region in the second to fourth decades of life, with a slight male predominance . Recent reports emphasize the importance of early genetic screening for PTCH1 mutations in recurrent or multiple OKCs, as they often represent the first sign of Gorlin–Goltz syndrome. Case: A 24-year-old male presented to our dental clinic with bilateral mandibular molar displacement and swelling of six years’ duration. Clinical and radiologic evaluation revealed multiple mandibular radiolucent lesions. After biopsy and subsequent genetic counselling, a diagnosis of Gorlin–Goltz syndrome (GGS) was established. The patient was followed for six years and underwent surgical excision of recurrent OKCs under general anaesthesia in 2019. Conclusion: Early diagnosis of Gorlin–Goltz syndrome is essential due to its association with multiple cystic lesions and potential neoplastic manifestations. Detection of multiple OKCs, often the initial sign of this syndrome, allows early intervention and may prevent more severe tumours in the future.