Simple SummaryPrimary ovarian leiomyosarcoma (POLMS) is a very rare malignancy characterized by unclear management and poor survival. We reviewed all 113 cases of POLMS reported in the literature till September 2022 to identify prognostic factors and the best treatment. Most patients received surgical resection, associated with lymphadenectomy in 12.5% of cases. Only 40% of patients received chemotherapy. POLMS is usually diagnosed at an early stage. Increasing stage and number of mitoses are associated with a worse prognosis. On the contrary, surgical resection with lymphadenectomy and chemotherapy is associated with increased survival. Ultimately, 43.4% of patients relapsed, and their mean disease-free survival was 12.5 months. There is a need for an international registry for POLMS that can help collect comprehensive and reliable data from around the world so that the best treatment can be definitively identified.AbstractBackground: Primary ovarian leiomyosarcoma is a very rare malignancy characterized by unclear management and poor survival. We reviewed all the cases of primary ovarian leiomyosarcoma to identify prognostic factors and the best treatment. Methods: We collected and analyzed the articles published in the English literature regarding primary ovarian leiomyosarcoma from January 1951 to September 2022, using PubMed research. Clinical and pathological characteristics, different treatments and outcomes were analyzed. Results: 113 cases of primary ovarian leiomyosarcoma were included. Most patients received surgical resection, associated with lymphadenectomy in 12.5% of cases. About 40% of patients received chemotherapy. Follow-up information was available for 100/113 (88.5%) patients. Stage and mitotic count were confirmed to affect survival, and lymphadenectomy and chemotherapy were associated with a better survival rate. A total of 43.4% of patients relapsed, and their mean disease-free survival was 12.5 months. Conclusions: Primary ovarian leiomyosarcomas are more common in women in their 50s (mean age 53 years). Most of them are at an early stage at presentation. Advanced stage and mitotic count showed a detrimental effect on survival. Surgical excision associated with lymphadenectomy and chemotherapy are associated with increased survival. An international registry could help collect clear and reliable data to standardize the diagnosis and treatment.