Review and Perspectives

Virchows Archiv

, Volume 460, Issue 3, pp 237-249

First online:

Ovarian carcinomas: five distinct diseases with different origins, genetic alterations, and clinicopathological features

  • Jaime PratAffiliated withDepartment of Pathology, Hospital de la Santa Creu i Sant Pau, Autonomous University of Barcelona Email author 

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Abstract

Malignant epithelial tumors (carcinomas) are the most common ovarian cancers and also the most lethal gynecological malignancies. Based on histopathology and molecular genetic alterations, ovarian carcinomas are divided into five main types (high-grade serous (70%), endometrioid (10%), clear cell (10%), mucinous (3%), and low-grade serous carcinomas (<5%)) that account for over 95% of cases. These types are essentially distinct diseases, as indicated by differences in epidemiological and genetic risk factors, precursor lesions, patterns of spread, and molecular events during oncogenesis, response to chemotherapy, and prognosis. For a successful specific treatment, reproducible histopathological diagnosis of the tumor cell type is critical. The five tumor types are morphologically diverse and resemble carcinomas of the uterus. Actually, recent investigations have demonstrated that a significant number of cancers, traditionally thought to be primary ovarian tumors (particularly serous, endometrioid, and clear cell carcinomas), originate in the fallopian tube and the endometrium and involve the ovary secondarily. This review summarizes recent advances in the molecular pathology which have greatly improved our understanding of the biology of ovarian carcinoma and are also relevant to patient management.

Keywords

Ovary Carcinomas Histopathological types Molecular genetics TP53 BRCA KRAS PTEN ARID1A HNF-1beta PIK3CA CTNNB1