Multiple odontogenic cysts in a patient with Neurofibromatosis–Noonan syndrome

Neurofibromatosis–Noonan syndrome (NFNS) is an uncommon chromosomal disorder showing features of both neurofibromatosis (NF-1) and Noonan syndrome (NS). We encountered a case of NFNS with keratocystic odontogenic tumor and dentigerous cysts. A 19-year-old Japanese girl was referred to our hospital with a chief complaint of swelling in the left side of the mandible. The patient showed symptoms of both NF-1, in the form of café-au-lait spots and neurofibromatosis, and NS, in the form of short stature, intellectual disturbance, webbed neck, and hypertelorism. Panoramic radiography showed three cystic lesions, one on each side of the mandible and one on the left side of the maxilla. All cysts were removed surgically under general anesthesia. Histopathological examination revealed that both mandibular cysts were dentigerous cysts, while the maxillary cyst was a keratocystic odontogenic tumor. No recurrence has been seen as of 5 years postoperatively. Multiple jaw cysts might be added as a potential finding in NFNS.