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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