Table 1.
Clinical features of bilateral WT
| AEIOP (Ref. 59) | SFCE (Ref. 64) | NWTS (Ref. 58) | JPLT (Ref. 62) | GPOH/SIOP9 (Ref. 65) | UKW2 (Ref. 60) | Netherlands (Ref. 56) | Egypt (Ref. 63) | Durban (Ref. 57) | Cape Town (Ref. 61) | Total | |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Number of patients | 93 | 49 | 188 | 31 | 28 | 70 | 25 | 22 | 20 | 19 | 545 |
| Date of study | 1990–2011 | 1993–2001 | 1986–1994 | 1996–2011 | 1989–1994 | 1980–1995 | 1967–2007 | 1993–2008 | 2002–2012 | 1981–2003 | 1967–2012 |
| Median or mean age | 24 m [5–86] | 2.3 y [1.7 m-8.4y] | 32 m [1–127] | 15 m [7–62] | 1.9 y | 24.4 m [1–102] | 1.03 y [0.27–5.35] | 3y [1–9] | 2.5y [10 m-9y] | 3.6 y [0.6–7.9] | |
| Sex | 32 M 61 F |
18 M 31 F |
74 M 114 F |
16 M 15 F |
28 M 42 F |
7 M 18 F |
10 M 12 F |
11 M 9 F |
7 M 12 F |
203 M 314 F |
|
| BWS | 3 | 0 | 10 | 0 | 2 | 2 | 0 | 0 | 17 | ||
| DD | 1 | 4 | 0 | 2 with WT1 mutation | 3 | 3 | 1 | 0 | 14 | ||
| WAGR | 2 | 3 | 1 | 0 | 5 | 0 | 0 | 0 | 11 | ||
| Isolated aniridia | 2 | 1 | 6 | 0 | At least one with 11p13 deletion | 0 | 0 | 0 | 0 | 7 | |
| Isolated HH | 5 | 1 | 15 | 0 | 0 | 0 | 1 | 0 | 22 | ||
| Perlman | 1 | 1 | 0 | 0 | 2 | 0 | 0 | 0 | 4 | ||
| GU anomalies | 0 | 1 | 6 hypospadias 11 undescended testis |
3 hypospadias 4 undescended testis 1 horseshoe kidney |
15 | 0 | 1 hypospadias with undescended testis | 0 | 42 | ||
| Other | 1 Prune Belly 1 Mental and growth retardation |
0 | 0 | 1 familial WT | 0 | 0 | 0 | 0 | 3 | ||
| WT1 mutation | 21 (68%) | 21 | |||||||||
| S or Me | All S | All S | All S | All S | All S | All S | 14 S 11 Me |
19 S 3 Me |
14 S 5 Me |
506 S 19 Me |
|
| Metastatic | 11 (12%) | 5 (10%) | 16 (8.5%) | 2 (6%) | 3 (11%) | 9 (13%) | 2 (8%) | 0 | 4 (20%) | 1 (5%) | 53 (9.7%) |
M, male; F, female; m, months; y, years; BWS, Beckwith–Wiedemann Syndrome; DD, Denis-Drash; WAGR, Wilms, Aniridia, Genito-urinary malformations and mental Retardation; HH, hemi-hypertrophy; GU, genito-urinary; S, synchronous; Me, metachronous.