Table 2

Other CPS (non-sex chromosomal aneuploidy syndromes) associated with paediatric ovarian GCTs

CPSSubtype of ovarian GCTs (age of onset in years)Observed casesFirst case
Cowden syndromeDysgerminoma (7)1Cho et al, 200877
Gorlin syndromeDysgerminoma (15)1Hoorens et al,  201684
BRCA1 germline mutationsDysgerminoma (16)1Werness et al,  200078
Down syndromeDysgerminoma (11–17)4Gesmundo et al, 198879
Satgé et al,  200680
Hasle et al, 200081
Smucker et al, 199982
Noonan-like with CBL mutationMixed GCT (immature teratoma, dysgerminoma and YST) at 7 years, mature teratoma (14)1Hanson et al, 201483
  • CPS, cancer predisposition syndrome; GCT, granulosa cell tumour; YST, yolk sac tumour.