Primary Hepatic Leiomyosarcoma: Case Report and Review of the Literature

Abstract

Purpose: Primary mesenchymal tumors of the liver are rare clinical entities (less than 1% of liver tumors) of which sarcomas, including multiple subtypes, represent even rarer occurrences. Less than 70 cases of primary hepatic leiomyosarcoma have been reported in the literature. Case: A 65-year-old white man with cirrhosis secondary to hepatitis C infection presented to the ER complaining of a 2-week history of abdominal pain with increasing abdominal girth. Physical exam was significant for a diffusely tender distended abdomen with hepatomegaly and moderate ascites along with caput medusae and spider angiomata. Laboratory results on admission revealed an AST of 83 U/L, ALT of 34 U/L, INR of 1.4, total bilirubin of 2.1 mg/dL, and an alpha fetoprotein of 2.9 ng/mL. A CT scan showed 12 cm liver lesion with invasion in the right hepatic vein, inferior vena cava, and extending into the right atrium where the mass measured 4x6 cm and traversed the tricuspid valve. A liver biopsy showed that the lesion was a high-grade sarcoma of either metastatic or primary liver origin. He began radiation therapy but died after his second treatment. Autopsy confirmed a large high-grade primary leiomyosarcoma. Discussion: Primary hepatic leiomyosarcoma is second most common hepatic sarcoma after hepatic angiosarcoma. Unlike hepatocellular carcinoma, primary hepatic sarcoma is not characteristically associated with underlying cirrhosis or viral hepatitis. Tumor is uniformly aggressive and thus usually diagnosed at advanced stage with an average size of approximately 10 cm at presentation. Presenting symptoms are generally vague abdominal or constitutional and cholestatic pattern is common owing to tumor burden effect. Tumor markers (alpha-fetoprotein, CA 19-9, CEA) tend to be either “normal” or minimally elevated at most. Radiologic appearance is variable (single vs. multiple lesions, hypervascular to avascular, aberrant vascularity). Definitive diagnosis is made with biopsy with immunohistochemical staining; however fine needle aspiration (FNA) is generally insufficient. Microscopy demonstrates clusters of spindleshaped cells containing elongated, cigar-shaped nuclei with blunt ends characteristic of smooth muscle. Spindle cell variant of hepatocellular carcinoma (HCC) should be in differential. Spread of sarcomas is usually local initially and our case demonstrates a very characteristic pattern of aggressive localized spread. Complete surgical resection offers the only true hope for cure but only in smaller lesions. The most common chemotherapeutic regimen for soft-tissue sarcomas is the combination of doxorubicin and ifosfamide however prognosis continues to be dismal.