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  • Molecular characteristics a...
    Hollis, Robert L.

    Cancer letters, 02/2023, Letnik: 555
    Journal Article

    Ovarian carcinoma (OC) is an umbrella term for multiple distinct diseases (histotypes), each with their own developmental origins, clinical behaviour and molecular profile. Accordingly, OC management is progressing away from a one-size-fits all approach, toward more molecularly-driven, histotype-specific management strategies. Our knowledge of driver events in high grade serous OC, the most common histotype, has led to major advances in treatments, including PARP inhibitor use. However, these agents are not suitable for all patients, most notably for many of those with rare OC histotypes. Identification of additional targeted therapeutic strategies will require a detailed understanding of the molecular landscape in each OC histotype. Until recently, tumour profiling studies in rare histotypes were sparse; however, significant advances have been made over the last decade. In particular, reports of genomic characterisation in endometrioid, clear cell, mucinous and low grade serous OC have significantly expanded our understanding of mutational events in these tumour types. Nonetheless, substantial knowledge gaps remain. This review summarises our current understanding of each histotype, highlighting recent advances in these unique diseases and outlining immediate research priorities for accelerating progress toward improving patient outcomes. •Ovarian cancer comprises multiple distinct disease types.•Each ovarian cancer type has unique clinical and molecular features.•Substantial advances have been made in our understanding of rare ovarian cancers.•Molecular understanding has led to targeted therapies for some ovarian cancers.•Significant gaps remain in our understanding of rare ovarian cancers.