Treatment of Keratocystic Odontogenic Tumors in Nevoid Basal Cell Carcinoma Syndrome

Nonsyndromic multiple odontogenic keratocysts: A case report.

Multiple Odontogenic keratocysts (OKCs) are one of the most frequent features of nevoid basal cell carcinoma syndrome (NBCCS). NBCCS is a genetic disorder transmitted by an autosomal dominant gene with variable expressivity, which is important to recognize when a patient has multiple OKCs. It is linked with mutation in the PTCH gene. Partial expression of the gene may result in occurrence of only multiple OKC. We hereby report a case of multiple OKCs in a non – syndromic patient and highlight the general practitioner the importance of diagnosing the disease and enforcing a strict long-term follow-up whenever such a case is identified.

Non-syndromic multiple odontogenic keratocysts: report of case / Keratocistos múltiplos não-sindrômicos: relato de caso

Odontogenic keratocysts (OKCs) are epithelial developmental cysts which were first described byPhillipsen in 1956. Lesions are frequently multiple and a component of Nevoid Basal Cell CarcinomaSyndrome (NBCCS) (Gorlin Goltz syndrome/Bifid rib syndrome). We hereby report a case of multipleOKCs in a non – syndromic patient and highlight the general practitioner the importance of diagnosingthe disease and enforcing a strict long-term follow-up whenever such a case is identified.

Os keratocistos odontogênicos são cistos de desenvolvimento que foram descritos primeiramentepor Phillipsen, em 1956. As lesões são frequentemente múltiplas, sendo componentes da síndromedo carcinoma nevoide de células basais (síndrome de Gorlin, síndrome das costelas bífidas).Descreve-se um caso de keratocistos múltiplos em paciente não-sindrômico, enfatizando-se aimportância do clínico geral no diagnóstico da doença e reforçando a necessidade de umapreservação a longo prazo tão logo a doença seja diagnosticada.

A Case Report of Multiple odontogenic keratocysts associated with basal cell nevus syndrome

The basal cell nevus syndrome is a well recognized entity, the major symptoms of which are basal cell nevi, multiple jaw cysts, skeletal anomalies, and ectopic calcification. The syndrome follows a hereditary pattern, which is characterized by a highly penetrant, autosomal dominant gene with multiple and variable effects. The patient often has a characteristic face, with frontal and temporoparietal bossing, which results in an increased cranial circumference. The eyes may appear widely separated, and 40 percent of patients have true ocular hypertelorism. Jaw cysts are one of the most constant features of the syndrome and are present in at least 75 percent of the patients. The cysts are odontogenic keratocysts and frequently multiple. Radiographically, the cysts in patients with basal cell nevus syndrome do not differ significantly from isolated keratocysts. The cysts in patients with this syndrome are often associated with the crowns of unerupted teeth; on radiographs they may mimic dentigerous cysts. We report a case of multiple odontogenic keratocysts associated with basal cell nevus syndrome with the literature of review.