Low-Grade Serous Carcinoma of the Ovary

Low-grade serous carcinoma is a rare tumor accounting for approximately 5% of ovarian carcinomas. It usually originates in a stepwise fashion from a serous borderline tumor. Its association with BRCA mutations is uncommon; however, it can be associated with KRAS, BRAF or ERBB2 mutations. The cardinal histological features of this neoplasm are the presence of mild to moderate cytological atypia and a mitotic index of up to 12 mitoses per 10 high power fields. Patients affected by disease tend to be younger and have a longer overall survival than those with high-grade serous carcinoma. Most cases present with advanced-stage disease. Surgery is the backbone of treatment aiming for a maximal cytoreduction. As this disease is not that sensitive to platinum-based therapy, the use of hormonal and targeted agents might be considered.