[Diagnosis and management strategies of hereditary ovarian cancer syndrome].
OBJECTIVE
To describe the clinical profile of hereditary ovarian cancer syndrome (HOCS), focusing on the diagnosis and management strategies.


METHODS
Eight patients from four families were identified with HOCS. The diagnosis was made by pedigree according to the criteria recommended by Lynch. Clinical manifestations and management strategies were discussed.


RESULTS
The incidence of epithelial ovarian cancer in HOCS family women was 20% which was higher than that in sporadic ovarian cancer (10%) (P < 0.05). The mean onset age in patients with HOCS was 41.6% years which was younger also than that in sporadic ovarian cancer. Serous cystadenocarcinoma was the predominant histological type of the HOCS, and the prognosis seems to be better.


CONCLUSIONS
Women in families with HOCS are in a population of high risk for epithelial ovarian cancer. Management strategies should include periodical counselling, screening and surveillance. For women with established families, above 40 years of age and identified with HOCS or with histories of breast or colorectal cancer, prophylactic oophorectomy should be considered.
Shen
K
K
Peking Union Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing.
Lang
J
J
Huang
R
R
D000328:Adult; D000368:Aged; D018284:Cystadenocarcinoma, Serous / Q000175:diagnosis* / Q000235:genetics / Q000517:prevention & control; D005260:Female; D006801:Humans; D008875:Middle Aged; D010051:Ovarian Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000517:prevention & control; D010375:Pedigree; D012307:Risk Factors