Angiosarcoma

Snapshot

A 75-year-old man presents to the clinic with complaints of generalized fatigue and weight loss. He states that over the past 3 years he lost a total of 15 lbs. without changes to his diet or his exercise. He notes that he can no longer copmlete his daily 2 mile walks because he would get extremely tired half way. Laboratory studies revealed mild normocytic anemia and mild elevations in transaminases. Physical examination reveals moderate tenderness to palpation at the right upper quadrant.

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
- Secondary Budd-Chiari syndrome
  - thrombosis of the hepatic vein that drain the liver
  - due to compression of the veins by angiosarcoma
Prognosis
- very poor prognosis as diagnosis is often very late in the disease process
mean survival time of 5 months

Presentation

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