Gorlin's syndrome with associated odontogenic cysts

Abstract

Multiple maxillary and mandibular cysts are principle features of basal cell nevus syndrome (Gorlin-Goltz). We present cases from an affected family in which magnetic resonance imaging (MRI) was helpful in evaluation of the cystic lesions. A middle ear anomaly was identified which may represent an additional abnormality associated with the syndrome.

Nevoid basal cell carcinoma syndrome: a 40‐year study in the South African population

“…With regard to gender distribution, there seems to be a wide range of data with some reports stating a male‐to‐female ratio of 1:1 while others stating a ratio of 3:1 (1, 5–7, 10, 12, 13) In this sample, the male‐to‐female ratio was 2:1, which confirmed the high prevalence of NBCCS in males.…”

“…Previous reports all agree that KCOTs occur more frequently in the mandible as compared to the maxilla and that the mandibular molar‐ramus region is the most commonly affected site (6, 7, 10, 14).…”

Expression of basement membrane components in odontogenic cysts

“…Multiple OKCs are the most common findings in naevoid basal cell carcinoma syndrome (NBCCS), occurring in 65–100% of patients (Lovin et al , 1991; Reisner et al , 1994). A clinical significance is that OKCs in NBCCS have been reported to more likely recur than sporadic OKCs (Woolgar et al , 1987; Mustaciuolo et al , 1989; Gorlin, 1995; Stoelinga, 2001).…”

Nevoid basal cell carcinoma syndrome: a review of the literature and a report of a case

“…Multiple OKCs are the most constant and common anomaly in NBCCS, occurring in 65–100% of patients, and usually appearing by the second or third decade of life [3,27]. The dentists’ diagnostic role surfaces when considering the fact that OKC development frequently antedates the syndromic basal cell naevi [2,7], thereby allowing an earlier diagnosis.…”