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Rupture with internal bleeding is not an uncommon complication of hepatic angiosarcomas that causes high morbidity and mortality. Transarterial embolization (TAE) may be effective for the temporary hemostasis of ruptured tumors. However, the blood supply of an angiosarcoma can also originate in the portal vein, and repeated internal bleeding may stem from this source. Elective liver resection following initial TAE is the standard curative treatment and is the only effective method for preventing further internal bleeding. High-risk liver resection is the only other choice for rescuing patients with fatal bleeding that cannot be controlled by TAE. We report a 66-year-old female patient with angiosarcoma who presented with repeated bleeding and developed hemorrhagic shock after the first episode of TAE. Following two more episodes of TAE, the patient developed internal bleeding with shock, but angiography revealed no arterial bleeding. Bleeding from the portal vein was highly suspected. To prevent further recurrent bleeding, salvage liver resection was subsequently performed under conditions of poor liver reserve. During the 4-month follow-up period until the patient died, no recurrent bleeding was noted. In conclusion, TAE is only a temporary treatment for internal bleeding from a ruptured hepatic angiosarcoma. Recurrent, potentially fatal bleeding may be caused by supplemental vascularization of the portal vein. Surgical tumor resection is necessary for the curative treatment of hepatic angiosarcomas and the prevention of repeated internal bleeding.

ResumenAntecedentesEl angiosarcoma hepático es un tumor vascular raro y maligno el cual representa el 2% de todos los tumores hepáticos primarios. La orientación diagnóstica es difícil, en especial cuando el paciente no tiene antecedente de exposición a carcinógenos relacionados como factores de riesgo. El diagnóstico es histopatológico, y en la mayoría solo se logra con autopsia ya que la toma de biopsia por aspiración con aguja fina conlleva riesgo de sangrado lo que limita su utilización.Caso clínicoMasculino de 41 años de edad el cual no tiene antecedentes de exposición a carcinógenos. Comienza con dolor abdominal secundario a hemoperitoneo por rotura tumoral y es diagnosticado por estudios de imagen con hemangioma hepático cavernoso gigante, tratado inicialmente con embolización y posteriormente con trasplante hepático. Seis meses después se presenta con hemoptisis por metástasis pulmonares. En la necropsia se reporta: angiosarcoma hepático metastásico.DiscusiónEl angiosarcoma hepático se ha relacionado con la exposición a carcinógenos, y continúa siendo una hipótesis su transformación a partir de una neoplasia vascular benigna. En el diagnóstico diferencial son útiles los estudios de imágenes, como la tomografía contrastada. Sin embargo, el diagnóstico definitivo es histopatológico con pruebas inmunohistoquímicas, que en el 35-100% solo establece en la autopsia.ConclusiónEs una neoplasia vascular maligna, donde la cirugía tiene un rol potencialmente curativo cuando se practica resección con márgenes libres. El trasplante hepático es controvertido ya que tiene pobre pronóstico a corto plazo.AbstractBackgroundHepatic angiosarcoma is a rare vascular malignancy that accounts for 2% of all hepatic primary tumours. The diagnosis is difficult, especially if the patient does not have history of exposure to carcinogens, which are considered as risk factors. The diagnosis is made by histopathology, but in a considerable percentage it can only be accomplished by autopsy. The performing of fine needle aspiration biopsy can lead to bleeding, with limitations in its use.Clinical caseA 41 year-old male, with no history of exposure to carcinogens, who developed abdominal pain secondary to a haemoperitoneum due to tumour rupture, was diagnosed by imaging methods with a giant cavernous hepatic haemangioma. He was initially treated with embolisation, and later with a liver transplant. After six months he developed haemoptysis secondary to lung metastasis. The autopsy reported metastatic hepatic angiosarcoma.DiscussionThis condition has been related to carcinogen exposure, with malignant transformation from a benign vascular neoplasia being proposed as a hypothesis. The differential diagnosis can be achieved with imaging studies such as CT scan, and the definitive diagnosis is made by histopathology with immunohistochemistry tests, with 35%-100% being made in the autopsy.ConclusionHepatic angiosarcoma is a malignant vascular neoplasia, the potential curative option is surgery with tumour free margins. Liver transplantation remains controversial because of its poor prognosis in the short term.

The haemangioma, the most common non-cystic hepatic lesion, most often discovered by chance, may in certain situations raise diagnostic problems in imaging. In this article, the authors first demonstrate that the radiological appearance of the hepatic haemangioma, in its typical form, is closely related to three known histological sub-types. They then show that certain atypical features should be known in order to establish a diagnosis. They also observe the potential interactions between the haemangioma, an active vascular lesion, and the adjacent hepatic parenchyma. Finally, they discuss the specific paediatric features of hepatic haemangiomas and illustrate the case of a hepatic angiosarcoma.

An hepatic angiosarcoma that developed 36 years after thorotrast administration is described with radiological and autoradiographic findings. An unusual feature of this case is the remarkably long latent period.

A retrospective epidemiological study of deaths from hepatic angiosarcoma (HAS) in the U.S. showed that during 1964--74 there were 168 such cases, of which 37 (22%) were associated with previously known causes (vinyl chloride, 'Thorotrast', and inorganic arsenic) and 4 (3.1%) of the remaining 131 cases with the use of androgenic-anabolic steroids. It is suggested that the long-term use of androgenic-anabolic steroids is the fourth cause of HAS, the majority of cases still being of unknown aetiology. Moreover, the presented cases serve as a link in a spectrum of hepatic disorders recently recognised to be caused by environmental agents such as vinyl chloride, arsenic, and thorotrast, and by contraceptive and anabolic steroids. Similar precursor stages, usually not recognised by clinical laboratory tests and consisting of areas of hyperplasia of hepatocytes and sinusoidal cells and sinusoidal dilatation, lead potentially to hepatic adenoma, carcinoma, peliosis, and angiosarcoma.

In a 45 year old man, hepatic angiosarcoma developed 33 years after medicinal arsenic ingestion of only six months' duration. In addition, the patient had a history of basal cell carcinomas without having had excessive sunlight exposure. Factor VIII staining of the tumor supports an endothelial cell origin of the tumor. The development of skin cancers and hepatic angiosarcoma in this patient suggests that there may be no safe threshold of arsenic exposure. A careful history concerning even short-term arsenic exposure in all patients with hepatic angiosarcoma may uncover more examples of this association.

The revolution in values in our society, symbolized by clusters of concepts often labeled “environment” and “participation,” creates expectations that did not exist only a few years ago. It is in this context that we must view the opinions said to be commonly held by workers regarding physicians and surgeons in the employ of industry (“company doctors”). Although they may have been long held, these opinions form the basis of an emerging crisis. The threshold for the acceptance or rejection of existing medical care related to the workplace has been altered radically by this revolution.

Dose-response data for the induction of angiosarcoma in rats exposed to various levels of vinyl chloride (VC) together with attendant biotransformation data were used to estimate the risk of developing angiosarcoma in persons exposed to VC. Since a biotransformation product of VC, not VC per se, is responsible for the induction of angiosarcoma, the body surface area of people relative to rats was used to estimate the dose of the carcinogen biotransformed from VC by the former. Four models were used to extrapolate the data. Using a probit model, 10 hepatic angiosarcomas were predicted to occur in a recently reported epidemiological cohort of 9677 workers whereas five have occurred. Linear models and that based on the equation, Risk = 1 − e−βx, where x = dose, do not appear as reliable. For an 8-hr day, 5 days/week, 35-year time-weighted-average exposure of 1 ppm, the predicted incidence of hepatic angiosarcoma using the probit model is 1.5 × 10−8.

A register of British cases of primary hepatic angiosarcoma (HAS) was established in 1974 to monitor the occurrence of cases from 1963 onwards. Details of cases dying in 1963-77 have been obtained. Thirty-five cases were agreed as HAS by panel of liver pathologists, and occupational and medical information was obtained in the majority of these. Two cases were attributable to VCM exposure, and eight others had received intra-arterial Thorotrast. In 1978-79, two more cases were confirmed in VCM polymerization workers.

A 64-year-old man developed hepatic angiosarcoma, and combined hepatocellular and cholangiocarcinoma, 36 years after Thorotrast administration. The patient presented with a large mass in the right upper quadrant. His serum alpha-fetoprotein increased from 800 ng/ml to 51.2 micrograms/ml, and liver biopsy disclosed hepatocellular carcinoma. At autopsy, the liver had two different malignant neoplasms; angiosarcoma and combined hepatocellular and cholangiocarcinoma. Metastases of angiosarcoma to both lungs and of cholangiocarcinoma to periaortic lymph nodes were also seen. Interestingly, some neoplastic cells of angiosarcoma exhibited globular hyaline inclusions, which were Periodic-Acid-Schiff reaction positive and diastase-resistant.

A case of hepatic angiosarcoma showed findings similar to those of cavernous hemangioma on dynamic CT, angiography, and magnetic resonance imaging. The tumor was histologically confirmed as angiosarcoma of cavernous pattern.

Hepatic angiosarcoma is a rare primary tumor of the liver with a mesenchymal origin. Diagnosis is difficult because clinical manifestations and imaging studies are inconclusive. In many cases a diagnosis is obtained during necropsy, not being apparent during the course of disease. It is associated with several risk factors, but these contribute to explaining only a few of all reported cases. When clinical manifestations begin progression is often fast, and possibilities for curative treatment are limited. We report two cases of hepatic angiosarcoma. In the first one, our patient had an insidious initial course, and then suddenly presented with hepatic failure followed by acute respiratory distress. A diagnosis was reached during necropsy. In the second case, we initiated the study of a chronic liver disease using fine-needle aspiration biopsy, which showed findings suggestive of hepatic angiosarcoma. In the following weeks the patient started on a torpid clinic course, and died from multiple organ failure.

Eleven cases of hepatic disease, including seven cases of hepatic angiosarcoma, have been identified to date among men employed at one vinyl chloride polymerization plant. The earliest diagnosis was made in April 1964. The two most recent cases, both angiosarcoma, were diagnosed in February 1974 as a result of systematic medical screening for liver abnormalities among workers at the plant. Ages at diagnosis have ranged from 36 to 58 years for the seven patients with angiosarcoma and from 28 to 56 years for the four patients with nonmalignant disease; durations of employment before diagnosis have ranged from 12 to 28 years and from 5 to 29 years. All 11 persons had worked in close and continuous contact with various phases of the vinyl chloride polymerization process. Review of pathologic material suggests the presence in both tumor and nontumor cases of portal fibrosis and atypical sinusoidal lining cells. A direct causal relationship between exposure to vinyl chloride monomer and pathologic findings is postulated. ( JAMA 230:59-63, 1974)

Recently, five cases of hepatic angiosarcoma were found in Louisville. All of the patients had worked in a plant engaged in the production of polyvinyl chloride (PVC) from vinyl chloride. A systematic health surveillance program was instituted to detect existing liver damage or angiosarcoma and to find early signs of developing liver damage. A protocol was devised that began with on-site phlebotomies done in the plant and progressed to sophisticated hospital studies, if important abnormalities were found. All of the 1,183 employees of the plant were tested. Two new cases of hepatic angiosarcoma and three cases of portal fibrosis were discovered by this program at the initial screening. It is hoped that continuous health monitoring will aid detection of liver abnormalities while still in a reversible or curable state. ( JAMA 230:64-68, 1974)

A 32-year-old woman with an eight-year history of oral contraceptive use was found at laparotomy to have a hepatic angiosarcoma. The prolonged exposure to contraceptive hormones may have been a factor in the development of this rare tumor. The estrogens and progestins contained in oral contraceptives are biochemically similar to the anabolic-androgenic steroids that have recently been reported to cause hepatic angiosarcoma. (JAMA1981;246:64-65)

Erythropoietin (Ep) dependent erythrocytosis was discovered in a 77-year old man with angiosarcoma involving the liver and spleen. At autopsy, tissue from tumorous and normal sections of liver was obtained and extracts prepared for Ep assay in exhypoxic polycythemic mice. Neither tumor nor normal extracts had Ep activity; however, when the extracts were incubated with normal plasma prior to assay, significant amounts of Ep were generated. The normal liver tissue had three times the activity of the tumorous portion, 0.21 U/ml. These results suggest that the tissue contained an Ep activator, probably erythrogenin. It is proposed that the greater amount of erythrogenin activity in the normal tissue may reflect an attempt at liver regeneration following damage by an invading tumor, as regenerating liver has been identified as a stimulus for extrarenal EP and erythrogenin production.

Hepatic angiosarcoma is a rare malignant vascular tumor, which accounts for up to 2% of all primary liver tumors. The most frequent symptoms on presentation are weight loss, weakness and abdominal pain. Diagnosis of diffuse hepatic angiosarcoma can be challenging. We report an original case of diffuse liver angiosarcoma revealed by haematological abnormalities initially diagnosed as an Evans syndrome. Anaemia and thrombocytopenia are rarely the first manifestations of this pathology. They are explained by combination of several mechanisms. Diagnosis of diffuse liver angiosarcoma can be extremely difficult and physicians should be aware of these presentation.