Liver angiosarcoma is a rare primary liver malignancy which originates from sinusoidal endothelial cells, accounting for approximately 1.8% of primary hepatic malignancy, affecting mostly men in their sixth or seventh decade of life. 

In this case report, a 77-year-old man presented with right upper abdominal pain and was initially investigated for urinary symptoms by his GP. The patient was found to have incidental liver lesion on CT-kidneys, ureters and bladder (KUB), which was revealed to be low grade angiosarcoma on biopsy results.

Background

Primary hepatic malignancies are divided into two groups: epithelial and non-epithelial. Non-epithelial tumours include hepatic angiosarcoma, rhabdomyosarcoma, lymphoma and haemangio-endothelioma.¹

Liver angiosarcoma is a non-epithelial mesenchymal tumour which usually presents with non-specific symptoms of anaemia, abdominal pain, abdominal distention, fever of unknown origin and weight loss.^2,3 Due to the aggressive nature of these tumours, they usually metastasise at the time of diagnosis.⁴ They can progress and cause fulminant hepatic failure.⁵

There is an association of angiosarcomas with environmental and occupational exposure to thorium dioxide, arsenic, radiation, vinyl chloride⁶ and diseases such as von Recklinghausen and haemochromatosis.⁷

Case report

This is a case of a 77-year-old man, who presented to the emergency department with a history of right upper abdominal pain, ongoing for the past six months and not associated with nausea, vomiting, jaundice or weight loss. His past medical history included chronic obstructive pulmonary disease (COPD), diverticulosis and tuberculous colitis in his 20s. He worked as a bar man, lived with his family, quit smoking 30 years ago and drank alcohol quite often. 

The patient denied any history of hepatitis or diabetes or family history of malignancies. He was not taking any regular medications and had no known drug allergies. A CT-KUB was carried out one week before admission to hospital. This was organised by his GP as part of work up for urinary tract infection. It showed a 1.2cm simple renal cyst in the upper pole of his right kidney and an incidental solid liver mass with surrounding smaller lesions in the right lobe of liver.

On physical examination, his abdomen was soft, mild tenderness was noted in the right upper quadrant but no viscera was palpable. Percussion elicited pain over the liver area. Cardiovascular and respiratory examination was normal. Laboratory results showed:

• WCC: 10 × 10⁹/L
• Haemoglobin: 12.6g/dL
• Platelet count: 227 × 10⁹/L
• Alanine aminotransferase (ALT): 20 U/L
• Aspartate transaminase (AST): 29U/L
• Alkaline phosphatase (ALP): 153 U/L
• γ-glutamyl transpeptidase (GGT): 214 U/L
• Albumin: 37 g/L
• Total bilirubin (TBIL): 6μmol/L
• Prothrombin time (PT): 11.0 sec
• International normalised ratio (INR): 1
• Alfa fetoprotein: Normal
• Carcinoembryonic antigen (CEA): Normal
• Ca 19-9: Normal
• Prostate specific antigen (PSA): Normal.

Subsequent work-up included CT-abdomen/pelvis with contrast, which showed a large heterogeneous liver mass involving the right lobe of the liver with irregular liver contour (see Figures A & B). Multiple hypodense smaller lesions of 5-10mm were noted in both lobes of the liver. Primary liver malignancy was suspected based on radiological images. CT-thorax and CT-brain were done as part of metastatic work-up but were normal.

Subsequent liver biopsy results showed a core of liver with complete effacement of the normal architecture by atypical vascular lesions with proliferation of vascular spaces lined by atypical endothelial cells (see Figures C & D). Immunostaining for the vascular endothelial markers including CD34 and Factor VIII were positive (see Figures E & F) plus SMA stain within the stroma was positive. The tumour was negative for D2-40. These features were highly suggestive of mesenchymal vascular lesion. Differential diagnosis includes epithelioid haemangio-endothelioma, kaposiform angiosarcoma and angiosarcoma.

The case was discussed in a hepatology multidisciplinary meeting in St Vincent’s Hospital, Dublin and details were sent to histopathologist in Brigham and Women’s Hospital, Boston, US for another opinion. Vascular lesions were confirmed as angiosarcoma. It was morphologically low grade and well differentiated.

Outcome and follow-up

Due to the aggressive nature of the tumour, surgery was not a viable option and it was recommended by the oncology team that chemotherapy would not offer any benefit for long-term survival. All options were discussed with the patient and he opted for palliative treatment.

The patient was admitted again with confusion and shortness of breath and haemoptysis. Liver functions were markedly deranged and a pulmonary embolism was found to be the cause of shortness of breath. The patient was treated by the palliative team primarily and he died within a few days of admission to hospital due to liver failure.

Discussion

Angiosarcoma is an aggressive malignancy of endothelial origin which commonly involves head and neck (60%).⁸ Liver angiosarcoma is more common in males with male to female ratio 3:1, mostly presenting with non-specific symptoms and liver function derangement, especially alkaline phosphatase seen in advanced stages of disease and thrombocytopenia.⁹ Due to vascular nature of the tumour they can easily rupture and present with intra-abdominal bleeding and peritoneal metastasis.¹⁰ Liver angiosarcomas were traditionally linked with occupational exposure to vinyl chloride, arsenic, thorium  dioxide and viral hepatitis, but a study in Taiwan found no major role of these carcinogens found in drinking water with incidence of hepatic angiosarcomas.¹¹

The diagnosis of hepatic angiosarcoma requires imaging studies and liver biopsy as tumour markers are usually within normal range.¹² Hepatic angiosarcomas are positive for vascular antigens including CD31, CD34 and mesenchymal vimentin.¹³

There are a few treatment options available for angiosarcoma, including tumour resection and liver transplantation. A recent study revealed that survival rates are very poor even after liver transplantation and it is contraindicated for diffuse hepatic angiosarcoma.¹⁴

Rapid progression, early metastasis, high recurrence rates and resistance to available chemo-radiotherapy results in poor long-term survival in primary hepatic angiosarcoma. However, palliative chemotherapy has shown improved survival in some cases.¹⁵ Most patients die within six months of diagnosis.¹⁶

References

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