Background: Cardiac tumors are a very rare entity. Leiomyosarcoma represents less than 1% of cases.
Case presentation: a 51-year-old woman diagnosed with primary left atrium leiomyosarcoma. She was treated by optimal surgery and adjuvant chemotherapy. She is still alive after a follow-up of 24 months without evidence of local or distant recurrence.
Conclusions: Cardiac leiomyosarcoma is a rare tumor with a dismal prognosis. Surgery is the mainstay of treatment. Adjuvant treatment is still controversial.
Keywords: Cardiac tumors; Chemotherapy; Leiomyosarcoma; Survival.