Onkológia 4/2019
Rare ovarian tumors
Ovarian carcinomas have long been on the first place in mortality of gynecological tumors. In two thirds of patients, the disease is diagnosed in advanced stages, which significantly impair the prognosis of the disease. The incidence of ovarian cancer increases with age and reaches the highest prevalence between 6 and 7 decades of life. The lifetime risk of developing ovarian cancer in the general population is around 1.5%. Genetic burden is described in up to 15% of cases. BRCA 1 mutation carriers are estimated to have a risk of developing ovarian cancer of about 39% and of BRCA 2 mutation carriers in 11-17%. Ovarian cancers are a very heterogeneous group of diseases. The tumor may be practically formed from any type of cell presented in the ovarian tissue. 90% of malignant tumors are epithelial carcinomas. Uncommon are gonadal mesoderm tumors and germinal tumors that affect younger women. All non-epithelial carcinomas represent a group of different biological behaviors, from benign to highly malignant forms, which represent a different principle in the therapy of these tumors. Most are treated with a multi-therapy approach, which may include surgery and chemotherapy, as well as other treatments such as hormonal therapy or targeted therapies. The biological nature of the individual subtypes can vary considerably, making it difficult to divide and review the entire range of these rare cancers. Different histomorphological and immunohistochemical types are a reflection of numerous molecular alterations, the knowledge of which is important not only for understanding the pathogenesis of these tumors, but especially for studying potential therapeutic options.
Keywords: ovarian cancer, non-epithelial carcinomas, gonadal mesoderm, germinal tumors, heterogenity
