Table 2.

Tumor risk in GS patients: main series from the literature

	Shanley [22] Australia	Kimosis [9] USA	Endo [10] Japan	Evans [4] UK
Number of patients	118	105	157	182
Variant identified				PTCH 126	SUFU 9	None 47
Median age at last follow-up	35	34.5	33.1	45	42	47
BCC*	90 (75%)	71 (80%)	56 (37.8%)	61 (48%)	4 (44%)	21 (45%)
KCOT	85/113 (75%)	78 (81%)	136 (86%)	79 (47%)	0 (0%)	16 (31%)
Medulloblastoma	1 (0.8%)	4 (4%)	4/120 (3%)	3 (2%)	3 (33%)	0
Meningioma	1 (0.8%)	2/42 (5%)		0	2 (22%)	2 (1.6%)
Ovarian fibroma	9/63 (14%)	9/52 (17%)	5/40 (12%)	4 (6%)	3 (43%)	4 (15%)
Cardiac fibroma			2/95 (2%)	2 (1%)	0	0

BCC Basal cell carcinomas. The definition varied according to authors: all BCC for Kimosis and Endo, > 10 BCC for Evans, multiple or before age 20 for Shanley

KCOT Keratocystic odontogenic tumors