Table 2.

Characteristics of gonadal tumor in type 1 Frasier syndrome (n = 45)a

Cases diagnosed Frasier syndrome by renal disorder or/and delay of secondary sexual characterb (n = 39)
No gonadal tumor (n = 15)Gonadoblastoma (n = 13)Dysgerminoma (n = 11)P
Age at gonadectomyYear (mean ± SD)Year (mean ± SD)Year (mean ± SD)0.519c
15.8 ± 4.813.6 ± 6.515.6 ± 3.8
WT1 mutationnnnP
 IVS9 −1A→G000
 IVS9 +1G→A001
 IVS9 +2T→C000
 IVS9 +4 C→T3220.775d
 IVS9 +5 G→A643
 IVS9 +6 T→A000
 No information675
Cases diagnosed Frasier syndrome by abdominal pain or mass (n = 6)
No gonadal tumor (n = 0)Gonadoblastoma (n = 3)Dysgerminoma (n = 3)P
Age at gonadectomyYear (mean ± SD)Year (mean ± SD)Year (mean ± SD)0.839c
NA11.7 ± 4.911.0 ± 2.0
WT1 mutationnnnP
 IVS9 −1A→G000
 IVS9 +1G→A000
 IVS9 +2T→C000
 IVS9 +4C→T0111.000d
 IVS9 +5G→A022
 IVS9 +6T→A000
 No information000
  • aCases with no information on age at gonadectomy and/or histologic features and/or diagnostic symptom (n = 26) were excluded from the total (73 cases) of type 1 Frasier syndrome in this table.

  • bOne case of type 1 Frasier syndrome with seminoma without information about WT1 gene mutation was excluded also excluded from this table.

  • cA P value was calculated by one-way ANOVA.

  • dA P value was calculated by the χ2 test.