Syndrome de Gorlin-Goltz : à propos d’un cas

Ghailan, Mohamed Rachid; Benhalima, Hanane; Rabeh, Ghafour; Oujilal, Abdelilah; Benbouzid, Anas; Bencheikh, Rachida Soulaymani; Essakali, L.; Boulaich, M; Kzadri, M.

doi:10.1051/mbcb/2007012

Cited by 2 publications

(1 citation statement)

References 3 publications

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“…In this syndrome, OKCs appear early in childhood and can be both numerous and voluminous. Therefore, some authors recommend that a genetic investigation be carried out to identify Gorlin-Goltz syndrome in patients with OKCs before the age of 20, which is when the OKCs are more likely to be numerous and/or when certain elements of the examination or interview point to developmental abnormalities, considering the predisposition to develop different cancers [4]. The genetic diagnostic examination must be considered in the light of arguments in favor of a syndrome.…”

Section: Discussionmentioning

confidence: 99%

A non-syndromic case of maxillo-mandibular keratocysts

Brechard

Hervé

Descroix

et al. 2020

J Oral Med Oral Surg

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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.

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Section: Discussionmentioning

confidence: 99%