Clinical study of hereditary ovarian cancer syndrome in Shandong province, East China

ObjectiveTo explore the clinicopathologic characteristics and prognosis of hereditary ovarian cancer syndrome (HOCS) in Shandong province, East China. MethodsThe present retrospective study assessed the clinicopathologic characteristics and prognosis among patients with HOCS treated at Shandong Cancer Hospital, China, between January 1, 2008, and January 31, 2016. Data from women with primary ovarian cancer who met HOCS diagnostic criteria (genetic-disease group) were compared with data from a control group of 100 women with sporadic ovarian cancer (sporadic-disease group) identified using simple random sampling. ResultsAmong 1247 records of primary ovarian cancer during the study period, 96 (7.7%) women from 77 pedigrees had HOCS (genetic-disease group), and 65 of these pedigrees had sufficient data available for inclusion. In the genetic-disease group, 55 (85%) and 10 (15%) women inherited the disease through the maternal and paternal lines, respectively. Age of onset was earlier in the genetic-disease group compared with the sporadic-disease group (P=0.016), the proportion of mucinous adenocarcinoma was lower in the genetic-disease group (P=0.006), and the genetic-disease group had higher 3-year (P=0.036) and 5-year (P=0.035) survival rates. ConclusionHOCS exhibited maternal lineage primarily, and featured an early age of onset, advanced disease (stage III), serous adenocarcinoma, and a better prognosis after comprehensive treatment (including surgery, chemotherapy, and occasionally radiotherapy) relative to sporadic ovarian cancer. Women with hereditary ovarian cancer syndrome experienced a better prognosis than women with sporadic disease; genetic counseling would provide diagnosis and treatment advice.