Rare Ovarian Tumors

Ovarian cancer is the eighth most common cancer among women globally. There are currently no feasible screening strategies for this disease. Diagnosis, treatment, surveillance, and survival of patients have improved over the years with advancements in radiology, pathology, genomics, and molecular biology. Individualized care incorporates precision surgery to limit morbidity. Germline genetic analysis and identification of somatic mutations in tumor tissue provide data for the use of targeted agents and immunotherapy. High grade serous carcinomas comprise 70% of diagnoses, but rare ovarian cancers affect women characterized by a wide spectrum of ages and risk factors. This chapter discusses the pathologic and molecular features of these cancers. The text also highlights the evolution of treatment to modern-day standards and the landmark trials that contributed to these changes.