Carcinosarcomas of the ovary, previously known as Malignant mixed Müllerian tumors (MMMT) of the ovary, are a rare type of mixed ovarian tumor with both epithelial and stromal components.
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Terminology
Carcinosarcomas of the female genital tract were previously known as malignant mixed Müllerian tumors to reflect the admixed histology comprising both epithelial and mesenchymal tissue. There is now increasing evidence to show that carcinosarcoma tumor cells instead demonstrate epithelial-mesenchymal transition. Thus the 5th edition of the WHO classification of female genital tumors (2020) recommends that the term "malignant mixed Müllerian tumor" is no longer used 7.
Epidemiology
They are very rare and account for less than 1% of all ovarian cancers. Most women are post-menopausal at the time of presentation and usually between their 6th to 8th decades of life.
Clinical presentation
Patients often present with an abdominal mass, ascites, and anemia. As with other ovarian tumors, the presentation can be vague.
Pathology
Carcinosarcomas are histologically biphasic tumors with both carcinomatous (epithelial) and sarcomatous (stromal) elements.
Radiographic features
On imaging, it is not possible to differentiate carcinosarcomas from other ovarian neoplasms; therefore, the diagnosis is essentially based on histopathologic findings. Features common to ovarian neoplasms, in general, may be present.
A high incidence of hemorrhagic ascites has been reported in those with advanced disease 6 - reported in >50% of patients with ascites from the tumors.
Treatment and prognosis
They are malignant, aggressive neoplasms 1,3 carrying a poor prognosis, even despite a combination of surgical debulking, radiation therapy, and chemotherapy. Metastasis generally involves serosal and peritoneal seeding. Median survival rates reported in the literature range between ~7-10 months from the time of initial presentation 5.
Staging
The FIGO system is commonly used for staging: see ovarian cancer staging.