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Updated: Dec 27 2021

Hepatic Angiosarcoma

  • Snapshot
    • A 69-year-old man presents to the emergency department with complaints of abdominal pain. The pain is concentrated at the right upper quadrant and is described as a dull ache. Computed tomography reveals several nodules in the liver. The patient reports that he used to work in construction before he retired.
  • Introduction
    • Overview
      • hepatic angiosarcoma is a rare, high-grade malignant vascular neoplasm
  • Epidemiology
    • Incidence
      • most common sarcoma arising in the liver
      • 0.1-2% of all primary tumors of the liver
    • Demographics
      • occurs in older patients (> 60 years of age)
      • more common in men
    • Risk factors
      • vinyl chloride
        • used to make polyvinyl chloride (PVC) pipes, wire coatings, plastic kitchen ware, and insulation
      • arsenic
      • anabolic steroids
      • radiation
      • thorium dioxide
    • Pathogenesis
      • majority of cases are of unknown etiology
      • malignant neoplasm of endothelial cells that line the walls of blood vessels of lymphatic vessels
        • can easily metastasize to distant sites
      • common sites of metastasis include spleen, lymph nodes, lung, bone, and the adrenals
    • Associated conditions
      • Budd-Chiari syndrome
        • thrombosis of the hepatic vein that drain the liver
  • Presentation
    • Symptoms
      • weight loss
      • jaundice
      • weakness
      • pain
        • RUQ or epigastric
        • constant
        • dull
    • Physical exam
      • hepatomegaly
      • palpable mass
      • distension (ascites)
  • Imaging
    • Computed tomography
      • best initial imaging study
      • findings
        • numerous poorly defined variably sized nodules
        • entire liver is frequently involved
  • Studies
    • Serum labs
      • often accompanied by thrombocytopenia
      • usually normal liver function until late in disease process
    • Tumor biopsy
      • immunostaining positive for vascular markers
        • ERG transcription factor
        • CD31
        • CD34
        • factor VIII antigen
    • Histology
      • grossly hemorrhagic and necrotic tissue
      • tumor composed of pleomorphic spindle or epithelioid cells, often with bizarre or multinucleated forms and mitosis
  • Differential
    • Hepatocellular carcinoma
      • histology appears as hepatocytes with differing degree of differentiation
    • Cholangiocarcinoma
      • laboratory studies often reveal cholestatic pattern
  • Treatment
    • Medical treatment
      • adjuvant chemotherapy as needed
    • Surgical and interventional
      • partial or complete hepatectomy
        • depending on presentation or location of tumor
      • liver transplantation
        • high recurrence rate
  • Complications
    • Liver failure
    • Intraabdominal bleeding secondary to liver rupture
  • Prognosis
    • Very poor prognosis as diagnosis is often very late in the disease process
    • Mean survival time of 5 months
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