Low grade serous ovarian cancer – A rare disease with increasing therapeutic options. (January 2023)

• Record Type: Journal Article
• Authors: Zwimpfer, Tibor A.; Tal, Ori; Geissler, Franziska; Coelho, Ricardo; Rimmer, Natalie; Jacob, Francis; Heinzelmann-Schwarz, Viola
• Is Part Of: Cancer treatment reviews. Volume 112(2023)
• Publication Date: 2023-01
• Subjects: ER -- KRAS -- CDK 4/6 -- MEK inhibitors -- BRAF inhibitors -- Endocrine treatment; Cancer -- Periodicals; Cancer -- Treatment -- Periodicals; Neoplasms -- therapy -- Periodicals; Cancer -- Périodiques; Cancer -- Traitement -- Périodiques; Cancer -- Treatment; Electronic journals; Periodicals; 616.99406
• DOI: 10.1016/j.ctrv.2022.102497
• Languages: English
• ISSNs: 0305-7372

Abstract

Graphical abstract: Highlights: LGSOC and HGSOC should be seen and treated as distinct ovarian cancer subtypes. LGSOC has a chemo-resistant nature and optimal treatment has not yet been defined. ER, KRAS, BRAF, CDK 4/6 and MAPK represent critical targets in LGSOC therapy. Endocrine treatment, MEK and BRAF inhibitors are promising treatment options. Results from ongoing clinical trials will probably change treatment regimens. Abstract: High-grade serous ovarian cancers (HGSOCs) most commonly arise from the fimbrial end of the fallopian tube and harbor TP53 gene mutations. In contrast, low-grade serous ovarian cancers (LGSOCs) appear to have different pathological, epidemiological, and clinical features and should be seen as a distinct serous epithelial ovarian cancer subtype. Our current understanding of LGSOC is limited, and treatment has generally been derived from the more common HGSOCs due to a lack of separate trial data. LGSOCs are characterized by slow tumor growth and are assumed to develop from serous borderline ovarian tumors as precursors. These cancers are often estrogen-receptor positive and show an activated mitogen-activated protein kinase pathway together with KRAS and BRAF mutations and, rarely, TP53 mutations. These characteristics are now commonly used to guide therapeutical decision making and, consequently, a substantial part of treatment consists of maintenance with endocrine treatment, thus balancing disease stabilization and mild toxicity. Additionally, new trials are ongoing that examine the role of targeted therapies such as MEK inhibitors in combination with endocrine treatments. The purpose of this work is to summarize current knowledge and present ongoing trial efforts for LGSOCs.