[Hereditary ovarian cancer as a separate clinical entity].

Family history of ovarian cancer has been regarded as the most important risk factor in developing this type of tumor. Recent identification of cloning of specific tumor suppressor genes responsible for hereditary ovarian cancer syndromes, resulted in the surge of enthusiasm and optimism regarding the practical application of genetic information. Many investigators believe that natural history of hereditary ovarian may significantly differ from sporadic cases. Some independent findings have shown that serous adenocarcinoma was the most frequent histological type in hereditary ovarian cancer patients, whereas the mucinous and the borderline were underrepresented in this group. Also, patients with hereditary ovarian cancer tend to develop the disease at younger than expected age. At the present moment, it remains unknown if environmental/lifestyle factors play a role in penetrance and expression of specific tumor suppressor genes in mutation carriers. The era of molecular genetics has raised hopes not only for a better understanding of biology and natural history of hereditary ovarian tumors but also a rapid breakthrough in prevention and management of this disease.