Carcinosarcoma is a mixed malignant biphasic tumour representing a rare entity and comprises of both epithelial and mesenchymal components. Primary ovarian carcinosarcoma is a rare neoplasm with a number of cases reported in the literature in the hundreds. It accounts for less than 1% of all ovarian tumours. These tumours are usually diagnosed at older age and advanced stage. It has aggressive clinical behaviour and survival depends on stage at presentation. Radiological imagings cannot differentiate carcinosarcomas from other ovarian cancers. Diagnosis is based upon histological findings. Cytoreductive debulking surgery is a crucial part in the treatment of carcinosarcoma of ovary. The role of adjuvant chemotherapy regimen is still controversial. Combination chemotherapy with taxane and platinum based regimen or ifosfamide and platinum based regimen are considered as adjuvant treatment. Despite aggressive treatment modalities such as surgery and chemotherapy, the outcome is poor. Response to therapy and overall survival for carcinosarcoma are poor in comparison to that of epithelial ovarian malignancies. Due to rarity of the disease, such poor prognosis needs collaboration of studies with molecular analysis to obtain new therapeutic guidelines to improve survival of the patients.
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