Sex: female
Age: 57 years
History
No exposure to Thorotrast, vinylchloride, or arsenicals.
No alcoholic abuse.
The patient started a diet 7 months ago with the intention of loosing 10 kg.
Laboratory data
Not available.
Physical findings
The patient seems to have a chronic disease. Splenomegalia and some hepatomegalia.
Case text
The patient presented with diffuse abdominal pain and increasing loss of appetite and a 20 kg weight loss
Image 1-4
Ultrasonography, including Doppler examination.
Axial view of the liver (image 1).
Sagittal view of the spleen (image 2).
Sagittal view of the liver, including Doppler (image 3).
Axial view of the liver, including Doppler (image 4).
Image 5-9
CT of the liver.
Axial CT scans, pre- and post-contrast enhancement. 10 mm slice thickness.
Post-contrast at a level near the diaphragm (image 5).
Pre-contrast at a level near the gastro-oesophageal junction (image 6).
Post-contrast at the same level as image 2 (image 7).
Post-contrast at the level of the caudal lobe (image 8).
Post-contrast at the level of the celiac trunk (image 9).
Image 10-12
MR of the liver.
Axial T2-weighted (TR/TE 1875/80) (images 10,11) and axial T1-weighted (TR/TE 600/20) (image 12) images.
Image 13-16
CT scan of the liver 2 months after the diagnosis, and after chemotherapy.
Axial, 10 mm slices at four different levels post-contrast.
Image 1-4
1. What are the abnormalities present on the ultrasonograms?
The echogenity of the liver is heterogenous. A 10 cm tumor centrally in the liver and multiple focal changes are seen. Portal hypertension with variable flow in the porta, gracile liver veins, and enhanced flow in the hepatic artery. Splenomegalia and varices.
Image 5-9
2. What are the abnormalities present on the CT scan?
Varices around the oesophagus and a dilated vena azygos. Enlarged spleen. Multiple focal areas in the liver with irregular borders. The tumor is hypodense pre-contrast, and with peripheral patchy enhancement post-contrast.
Image 10-12
3. What are the abnormalities present on the T1- and T2-weighted images of the liver?
The T1-weighted MR image shows a predominant intermediate-signal-intensity area occupying nearly the entire right lobe. High-signal-intensity foci within the tumor correspond with areas of hemorrhage. The T2-weighted MR image shows a tumor with a high-signal-intensity compared to normal liver tissue. Varices in the cardia area.
4. What is your diagnosis?
Angiosarcoma of the liver.
5. What is the differential diagnosis?
Hemangioma.
Hepatocellular carcinoma.
Vascular metastases.
6. What is the next step?
Follow-up imaging after chemotherapy.
Image 13-16
7. Comment on the evolutive aspects of the CT images, compared to set B?
The tumor has decreased in size. The tumor still has irregular borders and peripheral enhancement, varices and an enlarged spleen are still seen.
Final diagnosis
Angiosarcoma of the liver.
Differential diagnosis
- Hemangioma
- Hepatocellular carcinoma
- Vascular metastases
Discussion
Angiosarcoma of the liver is a rare tumor, although it is the most frequent mesenchymal malignancy in the liver. Angiosarcoma may be associated with previous exposure to toxics such as Thorotrast, vinylchloride, and arsenicals.
Because of the multi focal nature of the tumor and the propensity to metastasize, resective liver surgery or liver transplantation is usually not possible.
Due to the rarity of the tumor the responsiveness of angiosarcoma of the liver to chemotherapy or radiation is difficult to assess, although temporary regression can be seen.
Hepatic tumors with arterial-portal shunting may be the underlying cause of severe portal hypertension.