Smooth Muscle Surprise: Documenting a Primary Ovarian Leiomyosarcoma Case.

Primary ovarian leiomyosarcoma (LMS) is an extremely rare and aggressive tumor that originates in the smooth muscle tissue of the ovary. Typically affecting older women, it is diagnosed at an advanced stage and is associated with a poor prognosis, with most patients succumbing within the first year. The effectiveness of adjuvant chemotherapy and radiotherapy remains unclear due to the rarity of reported cases. In this case report, a 66-year-old woman presented with lower abdominal pain persisting for four months, accompanied by weight loss and appetite reduction. Imaging revealed a large, heterogeneous solid lesion in the left adnexa, suggesting a malignant ovarian neoplasm. Surgery was performed, and histopathological analysis confirmed the presence of a high-grade spindle cell sarcoma, consistent with ovarian LMS. Immunohistochemistry showed positive smooth muscle actin (SMA) and negative S100 and Myo D1, supporting the diagnosis. The patient completed two cycles of chemotherapy and was then lost to follow-up. This case highlights the diagnostic challenges and limited treatment options associated with primary ovarian LMS. Given the scarcity of cases, there is no established standard therapy, and the prognosis remains poor. Further research is needed to develop effective therapeutic strategies for this aggressive malignancy.