Low-Grade Serous Ovarian Cancer: Current Treatment Paradigms and Future Directions

Low-grade serous ovarian cancer (LGSOC) is a rare subtype of ovarian cancer, accounting for approximately 10% of cases of serous ovarian cancer. Patients typically present at a younger age have a protracted clinical course with survival for those with recurrent disease nearing 10 years, and have a high prevalence of somatic (tumor-specific) mutations affecting the mitogen-activated protein kinase (MAPK) pathway. Initial treatment of patients with stage IC-IV disease is similar to that of high-grade serous ovarian cancer with surgery and platinum/taxane-based chemotherapy. Selected patients may benefit from hormonal maintenance therapy following chemotherapy, in particular those with evidence of residual disease at completion of therapy. In the recurrent setting, the highest response rates to chemotherapy have been noted in those patients receiving chemotherapy in combination with bevacizumab. While hormonal therapies may offer disease stabilization with relatively low toxicity, objective response rates remain low. The use of targeted therapies such as MEK inhibitors remains an active area of investigation and those patients with MAPK pathway alterations may derive the greatest benefit from these agents.