Spotlight on carcinosarcoma of the ovary : a scoping review

Abstract

Ovarian carcinosarcoma, also referred as malignant mixed Mullerian tumour, is an uncommon, highly aggressive and malignant neoplasm which makes up 1 to 4% of all ovarian tumours. It is biphasic involving both malignant sarcomatous (mesenchymal) and carcinomatous (epithelial) cells. There are various subtypes such as serous and endometrioid. However, the mesenchymal part is sarcomatous. About 90% of cases of ovarian carcinosarcoma spread outside the ovary. The two most accepted theories of origin for carcinosarcoma of the ovary are the collision and conversion theories. A third theory is the combination theory. Prognosis remains poor even when still localised in the ovary. In the last few years, there has been no change in the survival rate. The median survival rate is lower than 2 years. Clinical features mainly include lower abdominal pain and a palpable abdominal mass. Ovarian carcinosarcoma remains poorly understood and understudied. Being a rare tumour, elaborate therapeutic consensus is not available for ovarian carcinosarcoma. The main treatment involves cytoreductive surgery and then chemotherapy. The type of chemotherapy, role of radiotherapy and novel therapies need to be further studied. The main objective of this article is to review the current literature on carcinosarcoma of the ovary.