epidemiology

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The frequency of Gorlin-Goltz syndrome among the general population has been estimated to range from 1/57,000(2) to 1/164,000 (1). High incidence rates have been reported in Australia. About 0.5% of all patients who develop multiple basal cell carcinomata will have this syndrome (2). There is no gender bias in its frequency. Typically, diagnosis is made in between 20-30 years of age

Ref:

(1) G. Chenevix-Trench, C. Wicking, J. Berkman et al., Further localization of the gene for nevoid basal cell carcinoma syndrome (NBCCS) in 15 Australasian families: linkage and loss of heterozygosity. Am J Hum Genet 53 (1993), pp. 760-767

(2) J.E. Springate, The nevoid basal cell carcinoma syndrome. J Pediatr Surg 21 (1986), pp. 908-910.

Last reviewed 01/2018