Learning objectives
To define the hepatic epithelioid hemangioendothelioma (HEH) among the other primary vascular liver tumours.
To recognise the radiological appearanceof the tumour and toidentify some
imaging features that will narrow down thedifferential diagnosis.
Background
Primary vascular tumors of the liver in adult patients include hemangioma,epithelioid hemangioendothelioma,
angiosarcoma andhemangiopericytoma.
Some of these are benign and common,
like angioma,
others are rare and low-grade malignant tumors,
likeepithelioid hemangioendothelioma.
Angiosarcoma andhemangipericytoma are very uncommon and agressive lesions.
The hepatic epithelioid hemangioendothelioma (HEH)is developing from the vascular elements of mesenchymal tissue.
Liver involvement occurs most often as a primary tumour and has a behaviour between benign hemangioma and aggressive hemangiosarcoma with extra hepatic spread.
ETIOLOGY:
The exact causes have not been clearlyidentified.
PATHOLOGY:...
Imaging findings OR Procedure details
Imaging findings
Modalities: US,
CT (nonenhanced and enhanced),
MRI (T1,
T2,
Diffusion,
ADC,
T1C+ sequences) PET-FDG,
arteriography.
Ultrasound:
On ultrasoundHEH presents as multiples peripheral hypoechoic masses or mixed hypo-hyperechoic masses,
with a hypoechoic rim.
(Fig. 5, Fig. 6)
In diffuse lesions,
US shows areas of overall decreased echogenicity that contain,
in some cases,
calcified foci.
There is no correlation between sonographic pattern and the size of the lesion.
CT:
Unenhanced CTimages reveal nodular foci of decrease attenuation because of their myxoid stroma.
In diffuse lesions,...
Conclusion
Final diagnosis of HEH requires a tumor biopsy,
followed by histopathologic findings.The prognosis depends on whether extrahepatic metastases are present at the time of diagnosis.
Extrahepatic involvement includes the peritoneal lymph nodes,
omentum and mesentery and may be associated with calcification.
Thoracic disease (intrapulmonary or pleural),
cutaneous or intramuscular metastases may be observed in some cases.(Fig. 15)
In the absence of extrahepatic disease,
radical resection is an option,
liver transplantation being considered the best option if metastatic desease.
Some imaging findings are highly characteristic for...
References
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The lollipop sign: a new cross-sectional sign of hepatic epithelioid hemangioendothelioma.
Eur J Radiol 2006;59(3):460–464.
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Torreggiani WC,
Harris AC,
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Hepatic epithelioid hemangioendothelioma: sonographic,
CT,
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