Multiple odontogenic cysts in a patient with Neurofibromatosis–Noonan syndrome

Abstract

Neurofibromatosis-Noonan syndrome (NFNS) is an uncommon chromosomal disordershowing 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.2

Developmental odontogenic cysts with special focus on the occurrence of multiple cysts: a single-centre retrospective study from the Czech Republic

“…Besides the NBCCS, multiple OKCs were occasionally observed in other syndromes as well, such as in Ehlers-Danlos syndrome [18,19], oral-facial-digital syndrome, also known as Papillon-Leage and Psaume syndrome [20], mental-retardation overgrowth syndrome, also known as Simpson-Golabi-Behmel syndrome [21,22] and in Noonan syndrome [23]. Even a case simultaneously presenting an OKC and bilateral DC was reported [24]. Other multiple DOCs, such as multiple OOCs [25,26], BCs/LPCs [27], COCs [28,29], and GOCs [30,31] are very rare and, to the best of our knowledge, are not associated with any syndrome.…”

Non syndromic synchronous multiple odontogenic keratocysts in a western Indian population: A series of four cases

“…About 10% of patients have multiple OKCs (either metachronous or synchronous), and half of these patients have Naevoid basal cell carcinoma syndrome (NBCCS) ( 2 ). Other syndromes with which multiple OKCs have been described are orofacial digital syndrome (OFDS) ( 3 ), Ehler-Danlos syndrome (EDS) ( 4 , 5 ), Noonan syndrome ( 6 , 7 ) , Simpson-Golabi-Behmel syndrome ( 8 ). There is no specific laboratory test to diagnose NBCCS and the diagnosis is made clinically using the specific criteria suggested by the first international colloquium on basal cell nevus syndrome (BCNS) ( Table 1 ).…”