CASE 17167 Published on 22.03.2021
0

Liver angiosarcoma: a challenging approach to a rare liver tumour

Section

Abdominal imaging

Case Type

Clinical Cases

Authors

António Proença Caetano1,2, Tiago Bilhim1,2

1. Interventional Radiology Unit, Hospital Curry Cabral, Centro Hospitalar Universitário de Lisboa Central (CHULC), 1069-166, Lisbon, Portugal

2. Nova Medical School, Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisbon, Portugal

Connected authors
Patient

47 years, female

Categories
Area of Interest Abdomen, Liver ; Imaging Technique CT, MR
Download as PDF Print Show related cases Notify admin
Clinical History

47-year-old woman, otherwise healthy, presents to the primary physician with recent onset of intense abdominal pain in the right upper quadrant. Physical examination was unremarkable; lab work revealed mild anaemia (Hb 10 g/dL). Abdominal ultrasound showed a large lesion of the right liver lobe and magnetic resonance imaging (MRI) was performed.

Imaging Findings

Abdominal MRI showed a dominant mass occupying most of the right liver lobe, measuring 11 cm (Fig. 1), heterogeneous but mostly hyperintense on T2WI and hypointense on T1WI, with areas of higher intensity on T2WI and scattered hypointense spots that were suggestive of intra-tumoral haemorrhage and hemosiderin deposits, respectively. The lesion showed marked restricted diffusion on high b values and, after contrast administration, there were multiple hypervascular foci suggestive of intra-tumoral haemorrhage and progressive extensive globular filling of the mass. The remaining lesions were much smaller in size and showed similar characteristics. Alpha-fetoprotein levels were normal.

Biopsy of the main lesion was performed under ultrasound guidance, and the patient was discharged. Pathology was suggestive of liver angiosarcoma, recommending excision of a single nodule if the results were not concordant with clinical and imaging findings.

Two weeks later, patient presents to the emergency department with severe anaemia (6,3 g/dL). Emergency CT was performed and showed spontaneously hyperdense areas within the dominant mass, with progressive filling after intravascular contrast injection, compatible with active bleeding (Fig. 2). A second percutaneous biopsy was performed simultaneously with embolisation of the main lesion (Fig. 3a-c), to confirm the diagnosis and treat the bleeding. Pathology from both liver specimens obtained reported a liver angiosarcoma with sinusoidal growth and focal atypia.

Follow-up CT performed 1 month later showed massive tumour necrosis and no internal enhancement (Fig. 3d-e). Treatment included a paclitaxel regimen followed by doxorubicin, with poor tumoural response 6 months later.

Discussion

Liver angiosarcoma is a rare malignant tumour and represents less than 2% of primary liver tumours [1]. It is associated with thorium dioxide, arsenic powder and vinyl chloride, and is more frequent in the 6th and 7th decades in males [2]. Most cases, however, do not show any association with known aetiologic factors [3]. Angiosarcoma is characterised by rapid growth, and most patients suffer from chronic liver disease. Symptoms are variable and nonspecific and include weakness, weight loss and abdominal pain [4]. Alpha-fetoprotein and CA 19-9 levels are usually normal.

Diagnosis is challenging, even when a histological specimen is obtained due to variability of tumour composition within an individual lesion [5]. There is a high risk of bleeding from biopsy, and open liver biopsies or laparoscopy may be more accurate and safer. For patient safety and due to the high vascular nature of the lesion, the second biopsy was done in the interventional radiology suite simultaneously with embolisation of the bleeding tumour to confirm the histology diagnosis.

Main differential diagnoses include cavernous haemangioma, hepatocellular carcinoma, epithelioid haemangioendothelioma, hypervascular metastases, Kaposi sarcoma and hepatic peliosis [6]. Lesions have increased vascularity, which translates into increased i.v. contrast uptake in CT and MRI scans and they may show progressive and globular enhancement, much like a cavernous haemangioma. However, solitary liver angiosarcomas are uncommon due to their insidious course, and multiple haemangiomas are atypical [1]. In comparison with haemangiomas, enhancement is usually less than that of the aorta and generally does not follow a centripetal pattern. On T1-weighted sequences, there is decreased signal with focal areas of high signal intensity, suggesting intra-tumoral haemorrhage [7]. There is increased signal on T2-weighted sequences. Elevation of the apparent diffusion coefficient level has been described [8, 9].

The majority of patients have unresectable lesions at diagnosis, as was the case with our patient. Metastasis is also common at presentation, in the lungs, spleen or bone [10]. Not surprisingly, prognosis is poor, with a mean patient survival of 6 months without treatment, and even when treated only 3% of patients survive over 2 years.

Differential Diagnosis List
Liver angiosarcoma
Hepatocellular adenoma
Cavernous hemangioma
Liver neuroendocrine tumour
Hepatocellular carcinoma
Hypervascular metastasis
Hepatic peliosis
Final Diagnosis
Liver angiosarcoma
Case information
URL: https://www.eurorad.org/case/17167
DOI: 10.35100/eurorad/case.17167
ISSN: 1563-4086
License

This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

Figure 1

MRI – Fat-Saturated T2WI– dominant well-circumscribed right lobe liver mass, heterogeneous in nature, showing diffuse hyp
MRI – Fat-Saturated T2WI– dominant well-circumscribed right lobe liver mass, heterogeneous in nature, showing diffuse hyperintensity on T2-weighted imaging and several foci of signal void (probable hemosiderin deposits).
MRI – Fat-saturated T2WI – multiple hyperintense liver nodules and masses scattered in the right liver lobe. These nodule
MRI – Fat-saturated T2WI – multiple hyperintense liver nodules and masses scattered in the right liver lobe. These nodules are more homogeneous in signal when compared to the large tumour.
Diffusion-weighted imaging – b50 – restricted diffusion of the dominant right lobe liver mass at low b values, with hyper
Diffusion-weighted imaging – b50 – restricted diffusion of the dominant right lobe liver mass at low b values, with hyperintense foci which are suggestive of intra-tumoral haemorrhage and multiple dark spots, probably representing hemosiderin deposits.
Diffusion-weighted imaging – b800 – restricted diffusion of the dominant liver mass at high b values, with hyperintense f
Diffusion-weighted imaging – b800 – restricted diffusion of the dominant liver mass at high b values, with hyperintense foci which are suggestive of intra-tumoral haemorrhage and multiple dark spots, probably representing hemosiderin deposits.
MRI – Fat-saturated T1WI vibe DIXON – pre-contrast – heterogeneous dominant right lobe liver mass, mostly hipointense i
MRI – Fat-saturated T1WI vibe DIXON – pre-contrast – heterogeneous dominant right lobe liver mass, mostly hipointense in signal compared to the healthy liver parenchyma, with hyperintense foci, compatible with intra-tumoral haemorrhage.
MRI – Fat-saturated T1WI vibe DIXON – post-contrast – arterial phase – heterogeneous progressive enhancement of the d
MRI – Fat-saturated T1WI vibe DIXON – post-contrast – arterial phase – heterogeneous progressive enhancement of the dominant right lobe liver lesion, showing some pockets of globular filling at the periphery.
MRI – Fat-saturated T1WI vibe DIXON – post-contrast – venous phase – heterogeneous progressive enhancement of the dom
MRI – Fat-saturated T1WI vibe DIXON – post-contrast – venous phase – heterogeneous progressive enhancement of the dominant liver lesion, with more spread out enhancement of the filling pockets compared to the arterial phase
MRI – Fat-saturated T1WI vibe DIXON – post-contrast – late phase – heterogeneous progressive enhancement of the domin
MRI – Fat-saturated T1WI vibe DIXON – post-contrast – late phase – heterogeneous progressive enhancement of the dominant liver lesion, with filling of nearly two thirds of the mass in a globular fashion Enhancement is higher than that of healthy liver parenchyma.
Figure 2

Post-contrast CT – arterial phase. Predominantly hypodense right lobe liver mass shows heterogeneous enhancement with hyper
Post-contrast CT – arterial phase. Predominantly hypodense right lobe liver mass shows heterogeneous enhancement with hypervascular foci, suggestive of intra-tumoral haemorrhage.
Post-contrast CT-venous phase. Predominantly hypodense liver mass shows heterogeneous enhancement with hypervascular foci, su
Post-contrast CT-venous phase. Predominantly hypodense liver mass shows heterogeneous enhancement with hypervascular foci, suggestive of intra-tumoural haemorrhage. There is slow progressive filling of the mass in a globular fashion.
Post-contrast CT – arterial phase. Multiple heterogeneous hypodense liver nodules with hypervascular foci.
Post-contrast CT – arterial phase. Multiple heterogeneous hypodense liver nodules with hypervascular foci.
Figure 3

Pre-embolisation angiography performed at the celiac trunk shows multiple vascular blushes compatible with intra-tumoral haem
Pre-embolisation angiography performed at the celiac trunk shows multiple vascular blushes compatible with intra-tumoral haemorrhage, during injection.
Pre-embolisation angiography performed at the celiac trunk shows multiple vascular blushes compatible with intra-tumoural hae
Pre-embolisation angiography performed at the celiac trunk shows multiple vascular blushes compatible with intra-tumoural haemorrhage that persist after injection.
Post-embolisation selective angiography at the proper hepatic artery shows vascular exclusion of the haemorrhagic foci.
Post-embolisation selective angiography at the proper hepatic artery shows vascular exclusion of the haemorrhagic foci.
Post-contrast follow-up CT – arterial phase. Post-embolisation CT depicts massive tumour necrosis of the right liver domina
Post-contrast follow-up CT – arterial phase. Post-embolisation CT depicts massive tumour necrosis of the right liver dominant mass A smaller lesion is shown in the left lobe, with a hypervascular foci.
Post-contrast follow-up CT – venous phase. Post-embolisation CT depicts massive tumour necrosis of the right liver mass.
Post-contrast follow-up CT – venous phase. Post-embolisation CT depicts massive tumour necrosis of the right liver mass.