Primary hepatic Angiosarcoma is a rare tumour that accounts for about 0.1%–2% of all primary malignant liver tumours. [1-3]. They are classified under mesenchymal tumours, of endothelial cell origin, generally appearing during the sixth or seventh decade of life. [3,4]. Most patients have a variable clinical presentation, from asymptomatic to nonspecific symptoms mimicking other chronic liver diseases including abdominal pain, fatigue, weight loss, jaundice, and anorexia or may present with liver failure.[4]. Various environmental carcinogens, such as vinyl chloride, thorium dioxide, and arsenic, are known to cause primary hepatic angiosarcoma. [5]. Exposure to these chemicals, especially arsenic, may be a part of medicinal, industrial or even occupational. Arsenic in drinking water sources has also been postulated. Among the spectrum of liver lesions attributed to chronic arseniasis, the significant ones include hepatocellular carcinoma, angiosarcoma, cirrhosis, and hepatoportal sclerosis. [5,6]. Hepatoportal sclerosis (HS) is a condition characterized by portal hypertension without evidence of liver cirrhosis. [6-8]. However, chronic arsenic exposure leading to Hepatoportal sclerosis followed by Angiosarcoma is very rare.

Figure 1: Enlarged portal areas showing inconspicuous portal veins, periportal mega sinusoids and herniation of veins into the lobules.

A 38yr old male presented with incidentally detected cirrhosis of the liver, grade 2 oesophagal varices, splenomegaly and a family

Angiosarcoma is an uncommon, highly malignant tumor that forms multiple or less often solitary haemorrhagic masses. Primary hepatic angiosarcoma is rare and accounts for about 0.1%–2% of all malignant primary liver tumours. [1-3]. The clinical presentation may vary, from asymptomatic to nonspecific symptoms mimicking other chronic liver diseases or may present as liver failure. [4]. The predisposing factors include treatment with arsenic, injection of radioactive contrast medium Thorotrast, industrial exposure to vinyl chloride and other postulates. [5]^.

Arsenic exposure can cause acute toxicity or chronic toxicity and has carcinogenic properties as well. It has a long history of use as an intentional poison, in medicines, pesticides, and even environmental exposure that stems from the pollution of drinking water in certain geographic areas. The significant liver lesions attributed to chronic arseniasis include cirrhosis, hepatocellular carcinoma, angiosarcoma and hepatoportal sclerosis. [6]. Hepatoportal sclerosis (also known as noncirrhotic portal fibrosis, idiopathic portal hypertension, and Banti syndrome) is an idiopathic noncirrhotic presinusoidal intrahepatic portal hypertension due to fibrosis of the intima of the portal vein and its branches. It is characterized by splenomegaly, hypersplenism and portal hypertension. [7-10].

In our case, the patient’s history of arsenic exposure and subsequent development of Hepatoportal Sclerosis were considered predisposing factors. The family resided abroad, and later the possible source of arsenic exposure was postulated to be their drinking water source.

Microscopy, in this case, revealed areas of cavernous hemangioma, epithelioid hemangioendothelioma and diffusely infiltrating Angiosarcoma.

In literature, cases have been reported regarding,