Case report

A 44-year-old man, a chronic alcoholic with no significant medical history, was admitted with complaints of pain in the right upper quadrant of the abdomen, nausea and loss of appetite and weight for 4 months.

There was no abnormal finding on physical examination. Liver biochemistry and routine blood investigations were normal. Viral markers were normal. USG revealed evidence of chronic liver disease with a mass suggestive of hepatocellular carcinoma in the right lobe of the liver.

CT scan showed a 9 x 5.9 x 8.1cm heterogeneously enhancing mass lesion involving the right lobe of the liver (Fig. 1).

Figure 1

Figure 1: CT scan showing a heterogeneously enhancing mass lesion involving the right lobe of the liver

Tumour markers AFP and CEA were within normal range.

Right hepatectomy was performed and the post-operative period was uneventful (Fig. 2)

Figure 2

Figure 2: Resected gross specimen of the right lobe of the liver

Histopathology showed a poorly differentiated angiosarcoma with cavernoma formation (Fig. 3).

Figure 3

Figure 3: Histomicrograph of poorly differentiated angiosarcoma with cavernoma formation

Immunohistochemistry profile: CD 31, CD 34, factor 8 positive, confirmatory for angiosarcoma (Fig 4).

Figure 4

Figure 4: Immunohistochemistry profile confirmatory for angiosarcoma

Retrospectively there was no history of exposure to any of the known carcinogens for angiosarcoma.

On follow-up at 1 month and 3 months postoperatively the patient was asymptomatic. CT scan showed no evidence of focal lesion in the residual liver.

Discussion

Angiosarcoma is responsible for about 2% of primary liver tumors₁, and is considered to be the most common of the mesenchymal liver tumors. About 25 cases occur each year in the United States. Males (ratio 3:1) in their fifth or sixth decade are most often affected₂, but it can also occur in children.₃₄ It often presents with abdominal discomfort and distension, weight loss, and fatigue. On examination, the patient may have jaundice, hepatomegaly, and ascites.₅ Liver function tests are usually abnormal. Thrombocytopenia, microangiopathic haemolytic anaemia, and disseminated intravascular coagulation may also be present₆. There are no tumour markers.

CT images classically show multiple hypodense areas. After administration of contrast medium, the lesions become partly or completely isodense with the normal hepatic tissue.₇ On T2-weighted MRI imaging, there are areas of high signals with central regions of low signals. Liver biopsy is hazardous as it may cause severe haemorrhage₈. Macroscopically, ‘blood lakes’ may be seen as the tumor is angioinvasive. On histological examination, there are typical spindle-shaped hyperchromatic cells with nucleoli, which, on immunostaining, are positive for endothelial cells markers.₉

More than 30% of cases have been linked to exposure to environmental agents including Thorotrast® which was used as a radiological contrast agent in the past. Evidence of previous exposure is seen on a CT scan, and the latency period can be longer than 30 years.₁₀₁₁

Angiosarcoma is also associated with exposure to vinyl chloride, which is used in rubber and plastic processing₁₂₁₃. Again the latency period can be very long. The risk ratio is 400:1 compared to the general population₁₄ and may be due to increased frequency of p53 mutations in these people₁₅. The incidence in this group has decreased since the acceptable exposure level to vinyl chloride has been reduced.

Angiosarcoma has also been reported to be associated with exposure to arsenic₁₆, anabolic steroids₁₇, and oral contraceptives.₁₈ It has been reported in a previous haemangioma₁₉, with Von Recklinghausen’s neurofibromatosis₂₀, and in patients with certain hereditary conditions such as congenital hereditary lymphoedema (Milroy’s disease) ₂₁ and Von Hippel Lindau disease.₂₂

Detailed history from our patient failed to reveal previous exposure to any of the known carcinogens or predisposing medical conditions. Our case is one of the few reports of primary angiosarcoma reported in literature.

Correspondence to

Dr Gaurav Maheshwari Dept of Surgical Gastroenterology Jagjivanram Hospital, Mumbai Email: drgauravmaheshwari@gmail.com