Systemic (chemo)therapies for Rare Gynecological Malignancies

Abstract

Abstract Rare gynecological cancers included real heterogeneous groups of disease and are associated with both difficult challenges and wonderful opportunities. Over the past decade or so, we have made progress in this area through refinement of pathological criteria, hypothesis-generating observational studies, revisiting the indication of adjuvant chemotherapy, and, most importantly, enhanced understanding of the molecular characteristics of several of these subtypes facilitated by technological advances. Surgery remains the cornerstone of management for all subtypes. However, with the exception of malignant ovarian germ cell tumors, for which conventional chemotherapy has been extremely effective, all of the other subtypes discussed herein share the feature of relative insensitivity to standard systemic treatment. Thus, investigation into novel therapeutics is a priority. In France thanks to the GINECO group and the National Cancer Institut (Inca) developed a national network dedicated to rare Gyn cancer since 2010, we have took this opportunity to work within the GCIG network to develop clinical trials dedicated to rare Gyn cancers, to delineate international guidelines. Over the next decade, further progress will occur through a multi-pronged strategy, including well-designed preclinical investigations, elegant observational and database studies, registries, and innovative clinical trials conducted by national and international networks or consortia as umbrella trials dedicated to Gyn tumors drive by molecular abnormalities.