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Primary leiomyosarcomas of the ovary are very rare tumors with only few cases having been reported so far and represent less than 0.1 % of all ovarian malignancies. These are highly aggressive tumors and have a very poor prognosis. Pathogenesis of this tumor is still uncertain. These cases mostly present in peri- and postmenopausal women aged 45–60 years. Leiomyosarcomas are usually unilateral and grow to a very large size making wide excision often impossible. These tumors are well circumscribed, large and often with necrosis, hemorrhage, and cystic degeneration. Marked cellular pleomorphism and brisk mitotic activity are frequent findings in such tumors. Most patients present with advanced disease having distant metastases to lungs and liver at presentation. Overall 5-year survival is reported as only 20–30 %. Because of its extreme rarity and low survival rate, we present this case of ovarian neoplasm in a young woman of 27 years with disease-free survival of 47 months after surgery and adjuvant chemotherapy.

Prolonged Survival of a Young Female with High Grade Pleomorphic Leiomyosarcoma of Ovary Without Recurrence