SYNCHRONOUS ODONTOGENIC MYXOMA AND ODONTOGENIC KERATOCYSTS IN NEVOID BASAL CELL CARCINOMA SYNDROME: A CASE REPORT

Abstract

Nevoid basal cell carcinoma syndrome (NBCCS) is a hereditary condition characterized by a wide range of developmental abnormalities and a predisposition to neoplasms. An 11-year-old girl was referred for evaluation due to a lack of eruption of teeth 14 and 15. Her past medical history and physical examination were unremarkable. Imaging exams showed well-defined pericoronal radiolucencies associated with teeth 14 and 37, as well as an additional radiolucent lesion affecting the right mandibular body. Microscopic analyses of both pericoronal lesions proved to be odontogenic keratocysts (OKC). The additional mandibular lesion showed a proliferation of spindle and stellate cells in a predominantly myxoid stroma and numerous concentric basophilic calcifications, consistent with odontogenic myxoma (OM). The patient was referred for genetic counseling and the diagnosis of NBCCS was made. To the best of our knowledge, this represents the second case of synchronous OKC and OM in a patient with NBCCS. Nevoid basal cell carcinoma syndrome (NBCCS) is a hereditary condition characterized by a wide range of developmental abnormalities and a predisposition to neoplasms. An 11-year-old girl was referred for evaluation due to a lack of eruption of teeth 14 and 15. Her past medical history and physical examination were unremarkable. Imaging exams showed well-defined pericoronal radiolucencies associated with teeth 14 and 37, as well as an additional radiolucent lesion affecting the right mandibular body. Microscopic analyses of both pericoronal lesions proved to be odontogenic keratocysts (OKC). The additional mandibular lesion showed a proliferation of spindle and stellate cells in a predominantly myxoid stroma and numerous concentric basophilic calcifications, consistent with odontogenic myxoma (OM). The patient was referred for genetic counseling and the diagnosis of NBCCS was made. To the best of our knowledge, this represents the second case of synchronous OKC and OM in a patient with NBCCS.