Primary Hepatic Leiomyosarcoma Presenting as a Budd-Chiari-Like Syndrome

Abstract

Figure: Spiral CT scan of the abdomen and pelvis demonstrating a large heterogenous mass occupying the majority of the right hepatic lobe and extending into the IVC.Figure: MRI of the abdomen demonstrating a large cystic and solid mass centered in the right hepatic lobe which extends into the caudate lobe causing near occlusion of the intrahepatic IVC.Primary hepatic leiomyosarcoma is a rare malignant tumor arising from smooth muscle cells of either vascular, biliary, or ligamentous origin. Diagnosis is often delayed due to the tumor causing nonspecific symptoms and often remaining asymptomatic until mass effect is produced. Less than 50 cases have been described in the literature. A 60-year-old male with extensive travel history to developing countries initially presented to a medical facility in Guam with bilateral lower extremity edema, dyspnea, fever, and fatigue. This was attributed to an upper respiratory infection and he was given a course of antibiotics and subsequently developed a diffuse desquamating rash felt to be a drug allergy. He ultimately underwent CT of the abdomen and pelvis which revealed a 13.6 x 16.8cm right hepatic lobe mass with obstruction of the inferior vena cava (IVC). He left Guam and presented to our institution two days later. Due to leukocytosis and fever, the initial suspicion was still largely infectious with hepatocellular carcinoma also on the differential; however he had no history of cirrhosis, hepatitis C, or alcohol abuse. CT guided liver biopsy was performed and demonstrated nonspecific fibrotic and infarcted liver parenchyma. As infectious and tumor markers were unrevealing, a repeat biopsy was obtained. This revealed a poorly differentiated carcinoma without a definitive primary site. Further imaging studies to assess for additional lesions were unrevealing. After extensive discussion with surgical oncology and cardiothoracic surgery he underwent open right hepatic lobectomy with resection of tumor and repair of the IVC. The patient tolerated surgery remarkably well and was subsequently discharged home with plans for potential adjuvant treatment. Surgical pathology, reviewed by an outside institution, demonstrated epithelioid elements with regions of smooth muscle morphology supporting the overall impression of high-grade primary hepatic leiomyosarcoma. Hepatic leiomyosarcoma is an exceedingly rare liver tumor with a poor prognosis due to the characteristically delayed presentation due to nonspecific symptoms. This case is consistent with the presentation and disease course described by previous case reports in the literature. This is thought to be a primary leiomyosarcoma of the liver, most of which are thought to arise from the wall of major vessels. Surgical resection is the management of choice with the role of chemotherapy and radiation not well defined.