Angiosarcoma of the liver is a rare form of malignant vascular tumor originating from endothelial cells. The course of disease is characterized by abdominal pain, weight loss, weakness, jaundice of the skin and mucous membranes, the phenomena of hepatomegaly, ascites. Pathology is diagnosed by ultrasound, CT, MRI of the liver, based on biopsy and diagnostic laparoscopy. The optimal tactic for liver angiosarcoma is radical surgical removal of the tumor; radiation therapy is also performed, and for metastases – polychemotherapy.
ICD 10
C22.3
Meaning
Angiosarcoma of the liver (hemangioendothelioma, endothelioblastoma, angioblastic sarcoma) has an extremely aggressive course, which is manifested by its rapid invasive growth, destruction of the liver parenchyma, bile ducts and organ capsules, germination in the branches of the hepatic and portal veins, a tendency to metastasis. In gastroenterology and hepatology, angiosarcoma accounts for 1.8% of cases of primary liver tumors; it is mainly detected in the male population aged 60-70 years. Disease is 30 times less common than hepatocellular carcinoma (primary liver cancer).
Causes
The development of liver angiosarcoma is associated with the carcinogenic effects of certain chemical compounds and toxic substances: vinyl chloride, thorotrast, steroids, radium, arsenic and copper compounds. In persons who have had contact with thorotrast (thorium dioxide), a radioactive drug used in radiology until the middle of the last century, angiosarcomas account for about 23% of all liver neoplasms. Thorotrast can also cause cirrhosis of the liver.
Many years may pass between the action of hepatotoxic substances and the development of liver angiosarcoma. In some cases, there are disease associated with hereditary diseases – neurofibromatosis (Recklinghausen’s disease) and idiopathic hemochromatosis.
Pathological anatomy
Angiosarcoma develops from the endothelium of sinusoids – capillaries located in the lobules of the liver. Macroscopically, with angiosarcoma, the liver is enlarged, with fuzzy borders; it contains multiple nodes resembling cavernous hemangioma, with areas of hemorrhages. In 80% of cases, pathology metastasizes to the spleen, in 60% – to the lungs.
Symptoms
The manifestations are not very specific. The development of the tumor is accompanied by abdominal pain, discomfort and heaviness in the epigastrium or right hypochondrium, weight loss, weakness, poor appetite, fever, shortness of breath. Hepatomegaly, jaundice, hemorrhagic ascites are rapidly progressing, indicating cholestasis and liver failure.
There are changes in the biochemical tests of the liver, thrombocytopenia. The DIC syndrome characteristic of disease is due to the influence of the tumor on clotting factors. A quarter of patients develop massive bleeding into the abdominal cavity. Within a year after the diagnosis, distant metastases develop in the spleen, lungs, adrenal glands, bones.
Diagnostics
Early detection of angiosarcoma presents great difficulties. An objective examination reveals jaundice of the skin color, liver enlargement, sometimes – palpable volumetric formation, with auscultation, noises in the projection of the liver are heard.
According to ultrasound of the liver, angiosarcoma is represented by single or multiple hyperechogenic formations. With an overview radiography of the abdominal cavity, CT (MSCT), MRI, thorotrast deposits in the liver and spleen are determined. With this disease, the substance is located on the periphery of the tumor formation. In the absence of thorotrast deposition on tomograms, liver angiosarcoma is defined as an area of reduced tissue density with high-density inclusions (foci of hemorrhages). The high standing of the diaphragm dome is characteristic.
The diagnosis of angiosarcoma is confirmed by liver biopsy or diagnostic laparoscopy with immunohistochemical examination of a tumor sample, in which the marker of endothelial differentiation CD31 is determined. Morphological examination of biopsy or surgical material reveals cavernous sinuses lined with malignant anaplastic endothelium – fusiform cells with hyperchromatic nuclei.
Treatment
With operable angiosarcoma, liver resection is necessary. Chemoembolization of the liver arteries is used to reduce the size of angiosarcoma before resection or to slow the growth of an inoperable tumor. Primary hemangioendothelioma is sensitive to radiation therapy, therefore, radiotherapy is used for unresectable tumors. With recurrent form and distant metastases, polychemotherapy with cytostatics is indicated. The prognosis for liver angiosarcoma is disappointing: most patients die within the first year after the tumor is diagnosed.