[Clinicopathological characteristics of hereditary ovarian cancer syndrome].
OBJECTIVE
To explore the clinicopathological characteristics of hereditary ovarian cancer syndrome (HOCS).


METHODS
From Jan. 2000 to Jan. 2007, among 580 cases of primary ovarian cancer, 42 cases (hereditary group), who had a positive family history of ovarian cancer and met the diagnostic criteria of HOCS, were analyzed retrospectively. One hundred cases without a family history of ovarian cancer were enrolled randomizely as control group (sporadic group).


RESULTS
The incidence of HOCS was 7.2% (42/580). Forty-two cases associated tumors affected at least 2 successive generations in 31 families and affected 1 generation in 8 families. Eighty-seven percent (27/31) was from maternal lineage, while 13% (4/31) from paternal lineage. Earlier age of onset was significantly difference between two groups [(49 +/- 10) years vs. (55 +/- 10) years, P < 0.05]. There were 90% belong to serous adenocarcinoma in the hereditary group, while 84% in the sporadic group. There was statistical difference in the proportion of mucinous adenocarcinoma (0 vs. 11%, P < 0.05). The most common clinical manifestations were abdominal distention and anorexia (64% vs. 70%, P > 0.05), International Federational of Gynecology Obstetrics (FIGO) stage III (62% vs. 63%, P > 0.05) between two groups. Fourteen cases (33%,14/42) were previously untreated in the hereditary group, while 40 cases (40%, 40/100) in the sporadic group. There were 15 cases (36%, 15/42) underwent secondary surgery and 15 cases (36%, 15/42) underwent third surgery or more in hereditary group, while 50 cases (50%,50/100) and 27 cases (27%, 27/100) in the sporadic group. The mean number of chemotherapy cycles received in two groups was 13.3 and 11.8 (P > 0.05). The 3-year and 5-year survival rate in hereditary group were 73.6% and 54.9% respectively, compared with 47.4% and 21.2% (P < 0.05) in sporadic group.


CONCLUSIONS
Hereditary ovarian cancer mostly from maternal lineage are featuring in early age of onset, serous adenocarcinoma, advanced stage (stage III), and better prognosis after the comprehensive treated by cytoreductive surgery plus with chemotherapy.
Zhong
Yan
Y
Department of Gynecological Oncology, Shandong Cancer Hospital and Institute, Jinan 250117, China.
Sheng
Xiu-gui
XG
Ma
Zhi-fang
ZF
Ma
Yue-bing
YB
Liu
Nai-fu
NF
Chen
Yue-ting
YT
Gao
Rong
R
Wang
Ying-ying
YY
Sun
Li
L
D000328:Adult; D017668:Age of Onset; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001943:Breast Neoplasms / Q000235:genetics; D002945:Cisplatin / Q000008:administration & dosage; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D018284:Cystadenocarcinoma, Serous / Q000235:genetics* / Q000473:pathology* / Q000628:therapy; D005260:Female; D030342:Genetic Diseases, Inborn / Q000473:pathology / Q000628:therapy; D020022:Genetic Predisposition to Disease; D006801:Humans; D008875:Middle Aged; D009367:Neoplasm Staging; D010051:Ovarian Neoplasms / Q000235:genetics* / Q000473:pathology* / Q000628:therapy; D010375:Pedigree; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors
D003520:Cyclophosphamide; D002945:Cisplatin