Table 7 The risk of gonadal malignancy associated with various types of DSD
Risk group | Disorder | Risk of malignancy (%) |
|---|---|---|
High | Gonadal dysgenesis: complete/partial, mixed gonadal dysgenesis (+Y: intra-abdominal) | 15–35% |
PAIS, non-scrotal | 50% | |
Frasier | 60% | |
Denys-Drash (+Y) | 40% | |
Intermediate | Turner (+Y) | 12 |
17-BHSD deficiency | 28 | |
Gonadal dysgenesis (+Y), scrotal | ? | |
PAIS, scrotal gonad | ? | |
Low | CAIS | 2 |
Ovotesticular DSD | 3 | |
Turner (−Y) | 1 |