Ann Hepatobiliary Pancreat Surg.  2021 Nov;25(4):532-535. 10.14701/ahbps.2021.25.4.532.

Diagnosis and surgical treatment of a rare hepatic angiomyolipoma with internal hemorrhage

Affiliations
  • 1Department of Surgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea
  • 2Department of Pathology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea

Abstract

Although angiomyolipoma (AML) is commonly found in the kidney, its appearance in the liver is rare. The first hepatic AML was reported by Ishak in 1976. Since then, there have been various reports of AML. An AML is a tumour affecting adipocytes, muscle tissues, and blood vessels. Hepatic AML has been associated with tuberous sclerosis. Its spontaneous occurrence has also been noticed. It can have a varied presentation with some being asymptomatic, others presenting with a dull aching pain, while some with disastrous consequences due to rupture and torrential bleeding intra-abdominally. Herein, we present a case of a 47-year-old female with a large hepatic AML having an internal hemorrhage that caused changes in hepatic arteries. In our case, magnetic resonance imaging was unable to establish a diagnosis. Intraoperatively, AML caused dilatation and engorgement of vessels around the porta. Immediately post resection, vessel dilatation and engorgement were reduced on table. Another notable feature was that these changes caused no intra-operative or post-operative hemodynamic changes. We report a case of a huge hepatic AML with internal hemorrhage associated with perihepatic vascular changes having a successful surgical treatment.

Keyword

Angiomyolipoma; Hemorrhage; Tuberous sclerosis; Engorgement; venous

Figure

  • Fig. 1 Preoperative images. Huge cystic tumor or hemorrhage in the right liver on computed tomography (CT) (A), suspected mural nodules inside the cystic tumor on magnetic resonance imaging (B), engorged hepatic artery and huge tumor on angiogram (C), and persistent cystic and solid huge tumor on immediate preoperative CT scan (D).

  • Fig. 2 Intraoperative images showing engorgement of vessels at the porta hepatis (A) and slight reduction of engorgement after right hepatectomy (B).

  • Fig. 3 Histopathological slides of hepatic angiomyolipoma. Low magnification view shows well delineated mass containing adipose tissue and irregular large blood vessels (A). High power view shows three components of angiomyolipoma: adipose tissue, blood vessels, and epithelioid to spindle cells (B). Immunohistochemistry shows HMB45 expression (C) and smooth muscle actin expression (D) of tumor cells.

  • Fig. 4 Follow-up computed tomography scans at six months after surgery showing well regenerated remnant left liver (A) and a further reduction in the size of the left hepatic artery (B).


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