Hepatic angiosarcoma
Hepatic angiosarcoma is a rare malignancy but is still the third most common primary liver tumour. They have a variable appearance on both CT and MRI reflecting the pleomorphic histological nature. Prognosis is very poor, with survival uncommon beyond one year from diagnosis.
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Terminology
Hepatic angiosarcoma was previously known as Kupffer cell sarcoma.
Epidemiology
Hepatic angiosarcomas account for ~1% of primary liver tumours. Most commonly occurs in patients in their 60-70s with a male predominance (M:F = 4:1) 1-3.
Clinical presentation
Patients typically have non-specific abdominal symptoms and may present with an abdominal mass or hepatomegaly. Patients can also present with spontaneous rupture and haemoperitoneum 1,5.
Pathology
Hepatic angiosarcomas arise from malignant spindle cells of endothelial origin. Metastases are common at presentation, affecting lung and spleen.
Aetiology
Most arise spontaneously, although haemochromatosis and neurofibromatosis type 1 have also been associated.
Environmental exposure to Thorotrast, arsenic, radiation and vinyl chloride have been implicated as risk factors but such exposure is now rare.
Histology
Histology demonstrates varied appearances of solid nodules/masses or as sinusoidal or cavernous spaces.
Radiographic appearance
Hepatic angiosarcomas most commonly present as multiple masses but can occur as a single heterogeneous mass 2,4.
CT
- hypoattenuating masses (some may be hyperattenuating reflecting haemorrhage) on both non-contrast and contrast-enhanced CT
- nodular enhancement is common
MRI
Findings reflect the haemorrhagic nature of angiosarcomas with fluid-fluid levels often a feature 2:
- T1/T2: heterogeneous areas of high signal reflecting mixed tumour and haemorrhage
- T1 C+ (Gd): heterogeneous enhancement with progressive filling
Nuclear medicine
- avid on FDG PET-CT 5
Treatment and prognosis
It typically rapidly progresses with metastases and high recurrence rate although partial liver resection may be successful if it is confined to one lobe of the liver.
Prognosis is poor with hepatic angiosarcomas being resistant to chemotherapy and radiation therapy. Median survival is less than six months and survival beyond one year is rare.
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Hepatobiliary pathology
- depositional disorders
- infection and inflammation
- liver abscess
- hepatic hydatid infection
- cirrhosis
- hepatitis
- cholecystitis
- cholangitis
- malignancy
- liver and intrahepatic bile duct tumours
- benign epithelial tumours
- hepatocellular hyperplasia
- hepatocellular adenoma
- hepatic/biliary cysts
- benign nonepithelial tumours
- primary malignant epithelial tumours
- hepatocellular carcinoma
- hepatocellular carcinoma variants
- cholangiocarcinoma
- biliary cystadenocarcinoma
- combined hepatocellular and cholangiocarcinoma
- hepatoblastoma
- undifferentiated carcinoma
- primary malignant nonepithelial tumours
- haematopoietic and lymphoid tumours
- primary hepatic lymphoma
- hepatic myeloid sarcoma (hepatic chloroma)
- secondary tumours
- miscellaneous
- adrenal rest tumours
- hepatic carcinosarcoma
- hepatic fibroma
- hepatic Kaposi sarcoma
- hepatic lipoma
- hepatic mesenchymal hamartoma
- hepatic myxoma
- hepatic rhabdoid tumour
- hepatic solitary fibrous tumour
- hepatic teratoma
- hepatic yolk sac tumour
- inflammatory myofibroblastic tumour (inflammatory pseudotumor)
- nodular regenerative hyperplasia
- pancreatic rest tumours
- primary hepatic carcinoid
- benign epithelial tumours
- extrahepatic bile duct tumours
- extrahepatic bile duct cholangiocarcinoma
- hilar cholangiocarcinoma (Klatskin tumour)
- extrahepatic bile duct cholangiocarcinoma
- liver and intrahepatic bile duct tumours
- metabolic
- trauma
- vascular
- portal venous gas
- portal hypertension
- portal vein thrombosis
- arterioportal shunts
- hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome)
- Budd-Chiari syndrome
- passive hepatic congestion
- hepatic veno-occlusive disease
- hepatic infarction
- peliosis hepatis
- hepatic venous malformations (haemangiomas)