Primary ovarian leiomyosarcoma in a 72-year-old nulligravid and the 2022 provisional diagnostic criteria for primary ovarian leiomyosarcoma / Philippine Journal of Obstetrics and Gynecology

@#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.

Primary ovarian leiomyosarcoma in a postmenopausal woman diagnosed using immunohistochemistry / Philippine Journal of Obstetrics and Gynecology

@#Primary ovarian leiomyosarcoma is a very rare tumor which is most commonly seen in postmenopausal women. Primary ovarian leiomyosarcoma has a very poor prognosis, with less than 20% of patients being alive at 5 years. The case is a 51‑year‑old female who presented with a lower abdominal mass secondary to an ovarian new growth. An exploratory laparotomy, peritoneal fluid cytology, unilateral salpingo‑oophorectomy with malignant frozen section of affected ovary, which revealed malignant tumor, proceeded with surgical staging total abdominal hysterectomy contralateral salpingo‑oophorectomy, then proceeded to complete surgical staging with infracolic omentectomy, Jackson‑Pratt drain insertion was performed. Microscopic and immunohistochemical findings established the diagnosis of primary ovarian leiomyosarcoma. Surgery is the cornerstone of treatment, while the role of chemotherapy and radiotherapy is still not clear because substantial data are lacking. The prognosis of primary pure ovarian leiomyosarcomas is extremely poor and there is no established treatment modality for this rare type of tumor.