Malignant mixed müllerian tumors of the ovary

Müllerian mixed malignant tumors of the ovary constitute an infrequently encountered group of malignant ovarian neoplasms, which are highly aggressive and respond poorly to treatment. The relatively low number of reported cases and the difficulty of preoperative diagnosis make it difficult to ascertain the biology of these tumors.

These are mixed, mostly monoclonal tumors, and the predominance of the stromal component aggravates the prognosis. The clinical presentation of these tumors is similar to that of epithelial ovarian tumors, although they tend to manifest themselves at later stages of disease. There are no useful biochemical markers: imaging diagnostic methods (ultrasound, computed tomography, magnetic resonance imaging) do not provide specific data. The staging and primary treatment are always surgical. Survival improves when cytoreduction is satisfactory. Chemotherapy (platinum) can prolong survival, but there are no effective second-line treatments. Radiotherapy is of no help. The prognosis of this cancer is always guarded. Genetic and molecular techniques will be very important in advancing our knowledge of tumoral biology. In order to improve therapeutic results, it will be necessary to design multicenter, cooperative studies including larger numbers of patients.

In clinical practice, treatment options for these tumors are few; a rapid downhill course is the rule rather than the exception.