Abstract
Primary hepatic leiomyosarcoma (PHL) is a rare malignant tumor, which originates from smooth muscles. Clinical presentation and imaging features are non-specific and can mimick the most frequent primary liver tumors namely hepatocellular carcinoma and intrahepatic cholangiocarcinoma. We report here two cases of PHL including one from the portal vein. The literature was searched for studies reporting cases of PHL reported from 2011 and 2019. The two patients were operated with R0 resection. Diagnosis of PHL was confirmed by histopathological and immunohistochemical examinations. Surgery remains the mainstay of the management of PHL. R0 resection is the main prognostic factor. Our literature search identified 16 additional cases from 12 reports. Preoperative diagnosis of PHL needs a high degree of suspicion due to atypical clinical presentation and non-specific imaging features. Surgery is the mainstay of the management of PHL. R0 resection is the main prognostic factor.