A non-syndromic case of maxillo-mandibular keratocysts

Abstract

Introduction: Odontogenic keratocysts (OKCs) are frequent, aggressive lesions with a strong tendency to recur, particularly in their para-keratinized majority form. Although they are mainly non-syndromic, these lesions are found in a large majority of patients with Gorlin syndrome. Thus, multiple forms are almost always associated with this syndrome and require investigation to prevent the risk of various cancers. Non-syndromic multiple forms are exceptional. Observation: A 20-year-old patient presented with dual localization of maxillary and left mandibular OKC at consultation. Under general anesthesia, excision of the lesions and extraction of the impacted wisdom teeth 28 and 38 were performed. The patient showed no clinical sign of Gorlin syndrome. Discussion: The OKC or epidermoid cyst is derived from the dental lamina or its remnants and from the basal part of the oral epithelium and represents between 10 and 20% of all cystic lesions in the maxillae. Its peak of incidence is between the second and fourth decade (or earlier in case of association with basal cell necrosis). OKC occurs mainly in the mandible and preferentially at the Ramus, where its frequency can reach 70% depending on the series. Conclusion: The management of OKC by oral surgeons must be conducted in a multidisciplinary setting in close collaboration with dermatologists, geneticists, and anatomic pathologists. Due to the strong recidivating character of OKCs, patient monitoring is essential.

A case of brachymetacarpia in a skeleton from a Mudejar cemetery from Spain (13th–14th century AD)

“…However, there are many other syndromes and diseases in which the presence of type E brachydactyly has been described, such as Gorlin‐Goltz syndrome (Brechard et al, 2020), Langer‐Giedion syndrome (N. Pereda et al, 2012), KBG syndrome (Libianto et al, 2019), brachydactyly mental retardation syndrome (Mahendhar et al, 2018), congenital constriction band syndrome (Satake et al, 2012), Turner syndrome (Page et al, 2017), microcephalic osteodysplastic primordial dwarfism (Bober & Jackson, 2017), and type E brachydactyly with associated hypertension (S.A. Temtamy & Aglan, 2008), lipid disorders (Page et al, 2017), and insulin resistance (Hyari et al, 2006). Likewise, it has been described more frequently in cases of short stature, without other anomalies (Sükür et al, 1997).…”

Odontogenic Keratocyst Mimicking a Radicular Cyst

“…Previously classified under developmental odontogenic cyst of the jaw by the WHO in 1971 and 1992, OKC was reclassified and renamed as keratocystic odontogenic tumor by the WHO classification of jaw tumors in 2005because of its aggressive nature and growth pattern and mutational changes. However, since 2017, the keratocystic tumor have again been classified as odontogenic keratocyst due to their pathogenic cystic process (2,3,4,5) Afterradicular and dentigerous cysts, odontogenic keratocystsare the third most common cyst andrepresent approximately 12-14% of all odontogenic cysts of the jaws. (1,6) OKC is mostly presented at the 2nd and 3rd decades and predominantly affect males.…”