Difficulty in Diagnosis of Leiomyosarcoma of Infrahepatic Inferior Vena Cava

Introduction

Leiomyosarcomas of the inferior vena cava are rare malignant and slow-growing tumors with a poor prognosis. It was first reported by Perl and Virchow in 1871, in the German literature. There were approximately 400 documented cases in the literature, which were originated from the smooth muscle cells of the media and predominantly proposed within the IVC. Resection was often presented with a challenge as these tumors may require reconstruction of the inferior vena cava (IVC). These papers reported on a case of surgical resection of an infrahepatic IVC leiomyosarcoma mimicking a hepatic tumor with IVC tumor thrombus.

Case Report

A 61-year-old woman was detected as her right upper abdominal in pain. Laboratory findings included that the total bilirubin of 3.8 mol/L, albumin 39.3 g/L, alanine aminotransferase (ALT) 46 IU/L, aspartate aminotransferase (AST) 98 IU/L, and alkaline phosphatase (ALP) 65 IU/L. She was HBsAg (-), HBV-DNA (-). Her alpha-fetoprotein (AFP), carcinoembryonic antigen(CEA)and carbohydrate antigen 19-9 (CA199) were all within the normal range. The functional status of the liver was assessed as Child A. Enhanced computed tomography of the abdomen revealed a tumor and was detected in the Spiegel of the liver with 7×6×5 cm mass. Areas of hemorrhage and necrosis may be noted within the mass on CT. Magnetic resonance imaging (MRI) of this tumor revealed a contrasting that low intensity on the T1-weighted image and high intensity on the T2-weighted image and extending from the left caudate lobe to IVC. We diagnosed this tumor is a hepatic tumor in the Spiegel lobe with IVC tumor thrombus.

A right subcostal laparotomy with upper midline extension to the xiphoid process was performed. A laparotomy was performed and intraoperative findings revealed a 7 cm×6 cm×5 cm hard- tumor involving the suprahepatic IVC. This tumor was not located in the Spiegel lobe of the liver but originated in the IVC. The falciform ligament was divided until the anterior surface of the suprahepatic IVC was exposed, and the infrahepatic IVC was dissected and mobilized. The tumor and the right renal vein was reached and exposed. In order to exposing the infrahepatic IVC lengthen, the left lateral section of liver was resected. The tumor was then dissected and removed from proximal to distal. After completed mobilization of tumor with adjacent pancreas, en bloc resection of the IVC tumor was performed under THVE (first Pringle’s maneuver, then the infrahepatic IVC occlusion with a Satinsky clamp, and last, suprahepatic IVC occlusion with a Satinsky clamp). The primary IVC wall was repaired longitudinally; and the relaxation was on the contrary. That was maintained for 10 minutes for the stage of enable resection. Intraoperative blood loss was 800 ml