ABSTRACT
@#Primary ovarian leiomyosarcoma (POLMS) is an exceedingly rare neoplasm accounting for only 0.1% of all ovarian malignancies and most commonly occurring in postmenopausal women. Prognosis is poor with only a 20% 5-year survival rate. Surgery remains to be its mainstay treatment. Discussed here is a 72-year-old nulligravid with hypogastric pain. Transrectal ultrasound showed a right ovarian new growth, probably malignant on International Ovarian Tumor Analysis (IOTA) simple rules, with a 79.2% risk of malignancy by IOTA ADNEX and an unremarkable uterus. Other workups were normal. She underwent primary cytoreductive surgery. Histopathologic diagnosis was ovarian leiomyosarcoma with positive immunohistochemical staining for desmin, S-100 protein, smooth muscle actin, and epithelial membrane antigen. She refused adjuvant chemotherapy postoperatively. The latest published literature on POLMS was also reviewed to develop the provisional criteria for its prompt diagnosis, thereby decreasing the heterogeneity of the diagnostic approach as well as supporting future researches on manifestations, clinical courses, and therapeutic plans.