0%
Topic
Review Topic
0
0
N/A
N/A
Questions
2 2
0
0
0%
0%
Evidence
2 2
0
0
https://upload.medbullets.com/topic/109088/images/angiosarcoma.jpg
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 
  • 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 
 

Please rate topic.

Average 4.3 of 4 Ratings

Questions (2)
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK

(M1.ON.13.7) A 58-year-old woman with a history of breast cancer, coronary artery disease, gastroesophageal reflux, and diabetes mellitus is diagnosed with angiosarcoma. Which of the following most likely predisposed her to this condition? Tested Concept

QID: 103800
1

Inherited dysfunction of a DNA repair protein

30%

(6/20)

2

History of exposure to asbestos

5%

(1/20)

3

History of chemotherapy

25%

(5/20)

4

History of mastectomy with lymph node dissection

35%

(7/20)

5

Hereditary disorder

5%

(1/20)

M 2 D

Select Answer to see Preferred Response

Evidence (3)
Topic COMMENTS (5)
Private Note