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Tania Moujaber Centre for Cancer Research, The Westmead Institute for Medical Research, Sydney, New South Wales, Australia
Faculty of Medicine and Health, The University of Sydney, Sydney, New South Wales, Australia
Crown Princess Mary Cancer Centre, Westmead Hospital, Western Sydney Local Health District, New South Wales, Australia
Blacktown Cancer and Haematology Centre, Blacktown Hospital, Western Sydney Local Health District, New South Wales, Australia

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Rosemary L Balleine Centre for Cancer Research, The Westmead Institute for Medical Research, Sydney, New South Wales, Australia
Faculty of Medicine and Health, The University of Sydney, Sydney, New South Wales, Australia
Children’s Medical Research Institute, Sydney, New South Wales, Australia

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Bo Gao Centre for Cancer Research, The Westmead Institute for Medical Research, Sydney, New South Wales, Australia
Crown Princess Mary Cancer Centre, Westmead Hospital, Western Sydney Local Health District, New South Wales, Australia
Blacktown Cancer and Haematology Centre, Blacktown Hospital, Western Sydney Local Health District, New South Wales, Australia

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Ida Madsen Centre for Cancer Research, The Westmead Institute for Medical Research, Sydney, New South Wales, Australia
Faculty of Medicine and Health, The University of Sydney, Sydney, New South Wales, Australia
Department of Gynaecological Oncology, Westmead Hospital, Western Sydney Local Health District, New South Wales, Australia

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Paul R Harnett Centre for Cancer Research, The Westmead Institute for Medical Research, Sydney, New South Wales, Australia
Faculty of Medicine and Health, The University of Sydney, Sydney, New South Wales, Australia
Crown Princess Mary Cancer Centre, Westmead Hospital, Western Sydney Local Health District, New South Wales, Australia

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Anna DeFazio Centre for Cancer Research, The Westmead Institute for Medical Research, Sydney, New South Wales, Australia
Faculty of Medicine and Health, The University of Sydney, Sydney, New South Wales, Australia
Department of Gynaecological Oncology, Westmead Hospital, Western Sydney Local Health District, New South Wales, Australia
The Daffodil Centre, The University of Sydney, a joint venture with Cancer Council New South Wales, Sydney, New South Wales, Australia

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Low-grade serous ovarian cancer (LGSC) is a morphologically and molecularly distinct subtype of ovarian cancer, accounting for ~10% of serous carcinomas. Women typically present at a younger age and have a protracted clinical course compared with the more common, high-grade serous ovarian cancer. Currently, the primary treatment of LGSC is the same as other epithelial ovarian cancer subtypes, with treatment for most patients comprised of debulking surgery and platinum/taxane chemotherapy. Primary surgical cytoreduction to no visible residual disease remains a key prognostic factor; however, the use of platinum-based chemotherapy in both upfront and relapsed setting is being questioned due to low response rates in LGSC. Most LGSC expresses steroid hormone receptors, and selected patients may benefit from endocrine maintenance therapy following chemotherapy, in particular, those with evidence of residual disease at completion of surgery. In the recurrent setting, while hormonal therapies may offer disease stabilisation with relatively low toxicity, objective response rates remain low. Strategies to increase response rates, including combining with CDK4/6 inhibitors, are being investigated. LGSC has a high prevalence of activating somatic mutations in mitogen-activated protein kinase pathway genes, most commonly in KRAS, BRAF and NRAS. Trametinib, a MEK inhibitor, has shown efficacy over chemotherapy and endocrine therapy. The use of combination targeted therapies, immunotherapy and anti-angiogenic agents, remain active areas of investigation for the treatment of LGSC.

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