Primary ovarian rhabdomyosarcoma: A diagnostic dilemma in an uncommon tumor.

Primary ovarian rhabdomyosarcoma (RMS) is an extremely rare and aggressive malignancy, with limited reported cases and no standardized treatment protocol. We present the case of a 17-year-old female who initially presented with progressive abdominal distension and was diagnosed with ovarian RMS. Imaging revealed a large solid-cystic pelvic mass, and histopathological evaluation, along with immunohistochemical markers such as desmin, myogenin, and WT1, confirmed the diagnosis. The patient underwent surgical resection, but recurrence occurred within 3 months, necessitating further evaluation. Early relapse suggested the aggressive nature of the tumor, requiring chemotherapy with VAC (vincristine, actinomycin D, and cyclophosphamide). This case highlights the importance of early diagnosis, and the urgent need for standardized therapeutic approaches to improve patient outcomes in ovarian RMS.