The authors present a review of pathogenesis, diagnosis and various approaches to treatment for an extremely rare tumor - primary hepatic leiomyosarcoma. This tumor is dif- ficult to be diagnosed by imaging examinations. The prevalent method of treatment is a radical resection (RO surgery). Ac- cording to literature the median survival of patients with LMSL is about one year with DFS after treatment about 10 months. Also the authors describe a case report of the successful cure of middle-aged female with primary hepatic leiomyosarcoma and Hodgkin's lymphoma in the anamnesis. She has undergone 6 cycles of neoadjuvant chemotherapy with Ifosfamide and Doxorubicin. The tumour response was estimated as partial. Then patient has undergone radical surgery in the volume of extended left hemihepatectomy with LND. Follow-up exami- nation 38 months after treatment revealed no progression of the disease.
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