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Primary leiomyosarcoma of the liver: Two new cases and a systematic review
Ann Hepatobiliary Pancreat Surg 2020 Feb;24(1):63-7
Published online February 29, 2020;  https://doi.org/10.14701/ahbps.2020.24.1.63
Copyright © 2020 Korean Association of Hepato-Biliary-Pancreatic Surgery.

Francesco Esposito1, Chetana Lim2, Laurence Baranes3, Chady Salloum4, Cyrille Feray4, Julien Calderaro5, and Daniel Azoulay4,6

1Department of General Surgery, Meaux Hospital, Meaux, 2Department of Hepatobiliary and Pancreatic Surgery and Liver Transplantation, Pitié-Salpêtrière Hospital, Paris, 3Department of Radiology, Henri Mondor Hospital, Créteil, 4Department of Hepatobiliary and Pancreatic Surgery and Liver Transplantation, Paul Brousse Hospital, Villejuif, 5Department of Pathology, Henri Mondor Hospital, Créteil, France, 6Department of Hepatobiliary and Pancreatic Surgery and Transplantation, Sheba Medical Center, Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
Received April 30, 2019; Revised May 5, 2019; Accepted May 9, 2019.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
 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.
Keywords : Leiomyosarcoma; Liver; Primary; Hepatectomy; Portal vein

 

February 2020, 24 (1)