Ann Hepatobiliary Pancreat Surg.  2020 Feb;24(1):63-67. 10.14701/ahbps.2020.24.1.63.

Primary leiomyosarcoma of the liver: Two new cases and a systematic review

• Esposito F
• Lim C
• Baranes L
• Salloum C
• Feray C
• Calderaro
• Azoulay D

• KMID: 2471191
• DOI: http://doi.org/10.14701/ahbps.2020.24.1.63

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.

Keyword

Leiomyosarcoma; Liver; Primary; Hepatectomy; Portal vein

MeSH Terms

Carcinoma, Hepatocellular
Cholangiocarcinoma
Diagnosis
Hepatectomy
Humans
Leiomyosarcoma*
Liver*
Muscle, Smooth
Portal Vein

Figure

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  Fig. 1 Leiomyosarcoma of the left portal vein. (A) Computed tomography. (B) Resected specimen. (C) Microscopic examination shows a tumor composed of atypical spindle cells with a fascicular growth pattern. Atypical mitoses were observed. (D) Immunohistochemical staining against alpha-smooth muscle actin was strongly positive.

• 

  Fig. 2 Giant leiomyosaroma of the right liver. (A) Computed tomography. (B) Magnetic resonance imaging. (C and D) Gross aspects on the resected specimen.

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