CASE 12199 Published on 26.11.2014

Hepatic epithelioid haemangioendothelioma (HEHE)

Section

Abdominal imaging

Case Type

Clinical Cases

Authors

Silviu BIVOL, Maïté LEWIN

Hôpital Paul Brousse
Service de Radiologie
12, avenue Paul-Vaillant-Couturier
94804 Villejuif Cedex
Email:maite.lewin@pbr.aphp.fr
Patient

29 years, female

Categories
Area of Interest Liver, Abdomen ; Imaging Technique CT, CT-High Resolution, Ultrasound
Clinical History
Abdominal pain in the right upper quadrant, fatigue, nausea.
Imaging Findings
Patient followed for liver and lung nodules of unknown aetiology.
Transabdominal US revealed multiple variable-sized hypo end hyperechoic lesions in both liver lobes.
Computed Tomography showed hepatomegaly and multiple hypoattenuating intraparenchymal lesions with slight central enhancement and more pronounced peripheral enhancement of these lesions after contrast administration.
Discussion
A. Background.
HEHE is a rare malignant liver tumour arising from vascular elements of mesenchymal tissue. It is more common in women in the fifth decade of life. The exact aetiology is unknown. A relationship with oral contraceptive use and vinyl chloride exposition has been suggested [3].
Pathology:
Two different types are described:
1) The nodular type
2) The diffuse type
HEHE is composed of two types of cells: dendritic or epithelioid, growing in preexisting vessels or neoformed vessels within a fibromyxoid stroma.

B. Clinical perspective.
Symptoms are nonspecific abdominal pain, weight loss, fatigue. Other symptoms include jaundice, fever, hepatomegaly, ascites or haemoperitoneum.

C. Imaging perspective.
Modalities: US, CT, MRI, PET-FDG, arteriography
US:
HEHE presents as multiples peripheral hypoechoic masses or mixed hypo-hyper echoic masses, with a hypoechoic rim.

CT and MRI:
Imaging of CT and MR are similar. Unenhanced CT images reveal hypoattenuating liver lesions.
After contrast administration, the tumour itself takes up a small amount of contrast medium in the central zone, although sometimes areas of hypervascularization can be detected. The tumour is enhanced in the periphery where a proliferating zone of active growth is present.
Central calcifications, areas of necrosis or haemorrhage can be seen on CT [1].
In the most advanced forms, confluent nodules are associated with the portal vessels invasion, signs of portal hypertension and non-tumoral liver enlargement.
The MRI reveals the target aspect of lesions: hypointense centrally with a peripheral thin hypointense rim in T1-weighted images, hyperintense centrally with a peripheral thin hypointense rim in T2-weighted images. After contrast administration, the target pattern is more evident, with three concentric layers [2]. Changes in hepatic contours are more evident in diffuse lesions, including capsular retraction. Portal vein tumour thrombus, obliteration of hepatic veins or sign of portal hypertension may be also encountered.

PET-FDG:
In some case reports, an increased fluorodeoxyglucose (FDG) uptake has been described.

Arteriography:
Hyper, hypo or avascular lesions

D. Outcome.
Liver transplantation is associated with favourable outcomes. Although liver resection and other local therapies may be reasonable choices in cases of small lesions, the presentation with multifocal disease often precludes the use of these treatments. Given the favourable outcomes after transplantation and the tendency for recurrence after liver resection, liver transplantation should be considered as a first-choice treatment.

E. Teaching points.
1. The capsular retraction adjacent to hepatic tumours. Many radiologists consider this sign to be associated with central cholangiocarcinoma but he was first described in HEHE.

2. The ”lollipop sign” is a characteristic radiological aspect which show the abrupt “cut-off” of the hepatic or portal vein [4].
Differential Diagnosis List
Hepatic epitheloid haemangioendotelioma
Haemangioma
Focal confluent fibrosis
Treated hepatocellular carcinoma or metastases
Cholangiocarcinoma
Metastases
Final Diagnosis
Hepatic epitheloid haemangioendotelioma
Case information
URL: https://www.eurorad.org/case/12199
DOI: 10.1594/EURORAD/CASE.12199
ISSN: 1563-4086