Primary perimenarcheal ovarian leiomyosarcoma

INTRODUCTION AND IMPORTANCE: Leiomyosarcomas of the ovary are extremely rare neoplasia usually occurring in perimenopausal patients. CASE PRESENTATION: A 16-year-old female patient, with no particular pathological history, who presented with chronic pelvic pain. On imaging; presence of a suprauterine solid cystic formation of 12 cm long axis. Tumor markers were normal. On exploration, solid cystic formation of 15 cm long axis. A total hysterectomy with bilateral salpingo-oophorectomy associated with bilateral pelvic and para-aortic lymphadenectomy and a total omentectomy were performed. Anatomical pathology; an ovarian leiomyosarcoma. CLINICAL DISCUSSION: Leiomyosarcomas of the ovary are extremely rare, representing less than 1% of all ovarian malignancies. They probably derive from the smooth muscle component. No definitive diagnostic criteria for ovarian leiomyosarcomas have been adopted to date, however the histological criteria adopted are those for uterine leiomyosarcomas. The differential diagnosis of these tumors includes fibrosarcomas, rhabdomyosarcomas, thecomas and extradigestive stromal tumors. The prognosis of ovarian leiomyosarcomas is generally poor. Complete surgical resection remains for all authors the cornerstone of treatment. The benefit of adjuvant therapies, namely chemotherapy or radiotherapy, remains to be proven. CONCLUSION: Leiomyosarcoma of the ovary, although exceptional, should always be included among the diagnostic possibilities when an ovarian cyst of organic appearance is discovered in a perimenarcheal patient.