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Table 1 Comparison of prevalence and frequency of major criteria in NBCCS patients among four studies.

From: Nevoid basal cell carcinoma syndrome (Gorlin syndrome)

  Evans et al. [5] United Kingdom, 1993 Shanley et al. [12] Australia, 1994 Kimonis et al. [6] United States, 1997 Lo Muzio et al. [11] Italy, 1999 Ahn SG et al. [14] Korea, 2004 Pruvost-Balland et al. [15] France, 2006
Patients 84 118 105 37 33 22
Prevalence 1/56,000 1/164,000 NR 1/256,000 1/13,939,393 NR
Major criteria % or (% African-Americans)
BCCs 47 76 80 (38) 30 15.2 100
   Age > 20 y 73 85 91 17 - -
   Age > 40 y 90 95 97 75 - -
Jaw cyst 66 75 74 92 90.9 62
Palm/plant pits 71 80 87 35 66.7 45
Calcification of falx cerebri NR 92 65 70 21.2 66
  1. NBCCS = Nevoid basal cell carcinoma syndrome; NR-Non reported