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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 
 

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Questions (2)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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(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? Review Topic | 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)

L 2 D

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