Hereditary ovarian/breast cancers (HOC/HBOC) Chair and Department of Surgical Gynecology and Gynecological Oncology of Adults and Adolescents. Head: Prof. zw. dr hab. n. med. Izabella Rzepka–Górska
HOC - SS HBOC (~80%)
Definitive criteria for HOC
at least 3 relatives with ovarian cancer one of them is I 0 or II 0 relative for two others
Criteria for high probability HOC
1/ two ovarian cancer diagnosed in I 0 or II 0 (any age)
2/ one ovarian cancer (diagnosed in any age) and patients second primary breast cancer or breast cancer diagnosed before the age of 50 years in I 0 or II 0 relatives
Clinical characteristics of HOC
I group HOC – pedigree criteria (patients with ovarian cancer)
II group HOC – patients with ovarian cancer and detected founder mutation of BRCA1 gene
Age at diagnosis (of ovarian cancer) HOC - 51,6 yrs HOC (BRCA1+) - 51,44 yrs
Age at diagnosis (of ovarian cancer) in Norway HBOC (BRCA1+) - 51,5 yrs Borg et al.
Age at diagnosis (of ovarian cancer) yrs (in general populations)
FIGO staging - III 0 & IV 0 (of ovarian cancer) HOC – 86,36% HOC (BRCA1+) – 83,87%
FIGO staging - III 0 & IV 0 (of ovarian cancer) in Norway HBOC (BRCA1+) - 81,82% Borg et al.
Morphological grading - G3 (of ovarian cancer) HOC – 81,25% HOC (BRCA1+) – 73,91%
Morphological grading - G3 (of ovarian cancer) in Norway HBOC (BRCA1+) - 82% Borg et al.
Histopathological type - serous (of ovarian cancer) HOC – 86,36% HOC (BRCA1+) – 80,77%
Histopathological type - serous (of ovarian cancer) in Norway HBOC (BRCA1+) - 91% Borg et al.
BRCA1 (ovarian cancer ) III/IV - 3
D N A testing
IHCC POLAND Head: Prof. dr habil. n. med. Jan Lubi ń ski 16 january BRCA1 Mutations Carriers The largest registry in the world
IN POLAND 13.5% of ovarian cancer develop in BRCA1 mutation carriers
Frequency of BRCA1 mutations in series of consecutive ovarian cancers from different populations POPULATION Frequency of mutations %n ASHKENAZI JEWISH35,5 27,5 67/189 57/208 POLISH13,549/364 PAKISTANI13,316/120 HUNGARIAN11,110/90 USA8,610/116 CANADIAN7,639/515 FRENCH CANADIAN5,15/99 FINNISH4,711/233 JAPANESE4,03/76 BRITISH3,412/355 NORWEGIAN3,32/60
Now we have the question?
What we can do with members hereditary cancer families ?
S c r e e n i ngS c r e e n i ng
1/ Intravaginal USG + CA beginning (yrs) Interval 6 (months)
Female genital tract screening by TV USG detects only 10% of early ovarian cancer NAROD S.A. ONTARIO 1999
What yet we can do?
2/ we recommend (patients with BRCA mutation carriers) Prophylactic adnexectomy and hysterectomy at the completion of childbearing > 40 yrs of age.
Prophylactic operations in our Clinic 1 january
I N C I D E N C EI N C I D E N C E
Every ovarian and breast cancer – indication for B R C A testing