Angiosarcoma of the liver poses a difficult diagnosis, due to its low incidence rate in comparison with other neoplasms, and its vague signs in the early stages [1]. It is a disease that is known to mainly affect elderly individuals but has also been documented in younger patients. This is especially the case in those with a history of exposure to vinyl chloride, which has been identified as one of the external factors associated with this cancer [2]. Symptoms such as general malaise, significant loss of weight and tiredness have been reported [3]. In contrast to the latter, another common presentation is a localized abdominal mass or generalized abdominal distension, in an otherwise clinically healthy patient [1]. Later stages of the disease usually display signs of long-standing liver disorders such as venous congestion, jaundice, anemia, coagulopathy, and hepatomegaly. This is the direct result of tumor expansion and development, involving an increasing portion of the liver and decreasing functional tissue [3]. Unexpected rupture of an angiosarcoma of the liver may lead to the initial presentation of shock, due to bleeding into the abdominal cavity, which in turn precipitates hypovolemia and cardiovascular collapse. Additionally, other signs and complications of hemoperitoneum may be experienced [4] [5].

Due to the tumor's propensity for metastasizing, there is a wide range of possible presentations, depending on the location of metastases. The latter can be intraabdominal, causing bleeding that is not from tumor rupture, blockage of blood vessels such as the portal vein, and organ dysfunction (commonly the spleen). Extraabdominal metastases can affect any organ system. Involvement of the lungs manifests as hemoptysis and respiratory distress. Likewise, the musculoskeletal system, bones, in particular, is often targeted [6].

• Easy Bruising

  The specific risk factors and cause of Cardiac Angiosarcomas are not well-established The signs and symptoms due to the tumor that is normally found in the right atrium, may include chest pain, anemia, and bleeding episodes (including easy bruising) In [dovemed.com]

• Splenomegaly

  A detailed review of 7 cases associated with one plant revealed that clinical features varied from little or no overt illness prior to diagnosis to advanced liver disease with portal hypertension and marked splenomegaly. [ncbi.nlm.nih.gov]

  In hindsight, this patient’s splenomegaly appears to be a result of periportal fibrosis and the splenomegaly and thrombocytopenia were long standing. [pagepress.org]

  The patient had not been previously diagnosed as cirrhotic and the imaging results showed a lack of splenomegaly, implying the absence of liver cirrhosis. [karger.com]

  Irregular liver surface, splenomegaly, and collateral circulation are noted, indicating cirrhosis. [bmcgastroenterol.biomedcentral.com]

• Fatigue

  Medications are often prescribed to reduce symptoms such as fatigue, anemia, and pains. Various surgeries are usually performed to promote a healthy appearance and a more comfortable day-to-day life for the patient. [knowcancer.com]

  Although the symptoms are usually nonspecific, abdominal distension and discomfort, weight loss, and fatigue are commonly found [ 6 ]. Jaundice, ascites and hepatomegaly are usually associated with advanced liver angiosarcoma. [npplweb.com]

  Some of these features include: Anemia Coagulopathy or clotting disorder Cardiac arrhythmias Loss of appetite Weight loss Collapse Pale skin Bone pain Fatigue Formation of lump or swelling under the skin Lymphangiosarcoma typically causes a bruise mark [hxbenefit.com]

  Males (ratio 3:1) in their fifth or sixth decade are most often affected 2, but it can also occur in children. 3 4 It often presents with abdominal discomfort and distension, weight loss, and fatigue. [ispub.com]

  Its symptoms are nonspecific and can include abdominal pain, weakness, fatigue, weight loss, anorexia, and abdominal enlargement. There is no relationship between hepatic angiosarcoma and portal hypertension. [surgicalcasereports.springeropen.com]

• Malaise

  Presenting clinical features included abdominal pain, malaise, jaundice, ascites, and massive hepatomegaly. In most cases the disease progressed rapidly, death occurring within a few weeks from hepatic coma. [ncbi.nlm.nih.gov]

  Symptoms such as general malaise, significant loss of weight and tiredness have been reported. [symptoma.com]

  Symptoms, which do not usually appear in its early stages, include weight loss, malaise, loss of appetite, abdominal swelling and pain, jaundice, fever, and ascites. When cancer has spread to another part of the body, pain can be felt there as well. [ugr.es]

• Dyspnea

  The child also experienced dyspnea on playing and had difficulty in moving about. There was no history of fever, jaundice, pain in the abdomen, vomiting or constipation. No history of delayed milestones or neurological symptoms was noted. [cancerjournal.net]

  HBV C spleen 9 W Factor VIII, UEA-I, vimentin, CD34 Tsai et al. [ 14 ] M 30 epigastralgia, general weakness None no none TAE+OP none 17 D NA Liang et al. [ 15 ] F 59 liver tumor none no none OP none 24 M Factor VIII, vimentin Tsai et al. [ 16 ] M 60 dyspnea [bmcgastroenterol.biomedcentral.com]

• Constipation

  There was no history of fever, jaundice, pain in the abdomen, vomiting or constipation. No history of delayed milestones or neurological symptoms was noted. The clinical differential diagnoses were malaria, hepatoma or viral hepatitis. [cancerjournal.net]

• Liver Mass

  Medical Illustrations Liver anatomy Liver imaging Liver lobectomy Journal Club May 2019 Bleeding Liver Masses: Imaging Features With Patho... Apr 2019 Expected and Unexpected Imaging Findings after 90Y... [ctisus.com]

  Tumor cells can also spread into adjacent blood vessels, into the abdominal cavity causing the accumulation of fluid (ascites) or masses elsewhere in the abdomen What Causes It Hepatocellular carcinoma most often develops in damaged livers. [cancersupportivecare.com]

  Hepatic mass and adjacent liver parenchyma with associated capsular disruption and inflammatory reaction (H&E stain, 40x magnification). [pagepress.org]

Presently, a combination of modalities is used to diagnose the condition. Imaging studies such as arteriography, which has been shown to be the most helpful, multiphasic contrast computerized tomography and magnetic resonance imaging scans are useful in confirming the suspicion of the disease. Imaging findings, however, are not always able to distinguish angiosarcoma of the liver from other hepatic malignancies [7]. To confirm the diagnosis, a liver biopsy needs to be done, frequently giving rise to many bleeding and fibrosis related complications thereafter. For this reason, open biopsies conducted in an operating theater, are preferred [8]. Despite these diagnostic measures, many cases are still diagnosed post-mortem.

Regarding necessary laboratory studies, liver function tests may be found to be normal or deranged, but they do not suggest a specific diagnosis. Similarly, tumor markers do not have a diagnostic role.

• Liver Biopsy

  Due to the vascularity of angiosarcoma, liver biopsy may be hazardous and can be avoided when there is a history of exposure to vinyl chloride monomer and the CT findings are consistent with the diagnosis. [ncbi.nlm.nih.gov]

  To confirm the diagnosis, a liver biopsy needs to be done, frequently giving rise to many bleeding and fibrosis related complications thereafter. For this reason, open biopsies conducted in an operating theater, are preferred. [symptoma.com]

  Conclusions Liver biopsies are difficult to obtain from patients with cryptogenic liver failure. [surgicalcasereports.springeropen.com]

  A portacaval shunt was performed, and liver biopsy yielded a diagnosis of cirrhosis. Repeat biopsies in October 1970 and September 1972 confirmed this diagnosis. Autopsy in March 1973 revealed angiosarcoma. [cdc.gov]

  Assessment of the diagnosis is difficult, particularly on percutaneous liver biopsy. [casereports.in]

After the onset of clinical symptoms, the disease often progresses rapidly, decreasing the chances of curative treatment. We report the case of an 83-year-old male patient who presented with postprandial fullness. [ncbi.nlm.nih.gov]

The third patient received conservative treatment only and survived for 42 months. [journals.lww.com]

The main aim of this treatment is to reduce the growth of the cancer or stop the cancer. In some cases, the treatment can extend the duration of life. [news-medical.net]

Prognosis - Angiosarcoma of the liver The prognosis of Angiosarcoma of the liver usually refers to the likely outcome of Angiosarcoma of the liver. [checkorphan.org]

Angiosarcoma is a rare primary malignant neoplasm of the liver with a poor prognosis. [ncbi.nlm.nih.gov]

Prognosis is poor with hepatic angiosarcomas being resistant to chemotherapy and radiation therapy. Median survival is less than six months and survival beyond one year is rare. [radiopaedia.org]

The survival rate is a standard factor to discuss the patient’s prognosis. The most complicated or severe prognosis of patients with angiosarcoma is death. [news-medical.net]

A case-control study indicated that direct exposure to arsenic, vinyl chloride (VC), and thorium dioxide was a significantly important factor in the etiology of this disorder (P less than 0.02). [ncbi.nlm.nih.gov]

Since the demonstration of an etiological relationship between hepatitis B virus infection and hepatocellular carcinoma, intensive research has been con ducted in an effort to elucidate the role of the virus in hepatocarcinogenesis. [books.google.com]

It arises from a number of known etiologies including both hereditary and acquired disease processes, as well as exposure to certain poisonous substances and industrial chemicals. The cause of most cases, however, remains unknown. [symptoma.com]

Etiology Most hepatic angiosarcomas arise spontaneously, although hemochromatosis and neurofibromatosis type 1 have also been associated. [radiopaedia.org]

For most diseases, a case of occupational origin is not clinically distinguishable from illness resulting from other etiologies. [cdc.gov]

Anon: Epidemiologic notes and reports. Angiosarcoma of the liver among polyvinyl chloride workers—Kentucky. MMWR 46(5)97-101, 1997 Google Scholar 7. [link.springer.com]

Publication of this report and a related journal article (5) stimulated a series of clinical investigations, epidemiologic studies, and toxicologic analyses. [cdc.gov]

View Article PubMed Google Scholar Mansell CJ, Locarnini SA: Epidemiology of hepatitis C in the East. Semin Liver Dis. 1995, 15: 15-32. 10.1055/s-2007-1007260. [bmcgastroenterol.biomedcentral.com]

Epidemiology Angiosarcomas are relatively rare. They comprise 1-2% of sarcomas in the surgical database from 1982-present from Memorial Hospital. Like other sarcomas, the risk of recurrence depends on the stage of disease. [curesarcoma.org]

References Zocchetti C: Liver angiosarcoma in humans: epidemiologic considerations. Med Lav. 2001, 92:39–53. Cano-Garcia F, Athie-Athie Ade J, Garcia-Gomez JI, et al.: Cystic angiosarcoma of the liver. A previously undescribed neoplasm. [cureus.com]

Prevention - Angiosarcoma of the liver Not supplied. Diagnosis - Angiosarcoma of the liver Not supplied. [checkorphan.org]

Liver cancer can’t always be prevented. However, you reduce your risk for liver cancer by taking steps to prevent the development of conditions that can lead to liver cancer. [healthline.com]

A SHE has been defined as "an unnecessary disease, disability, or untimely death whose occurrence signals a failure of prevention" (3). [cdc.gov]

There is no definitive method to prevent Primary Cardiac Angiosarcoma occurrence. [dovemed.com]

This is a rare and aggressive sarcoma and sarcoma patients should be closely watched by follow-ups, in order to prevent further complications after treatment. Microscope picture of an angiosarcoma. [news-medical.net]

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2. Fayette J, Martin E, Piperno-Neumann S et al. Angiosarcomas, a heterogeneous group of sarcomas with specific behavior depending on primary site: a retrospective study of 161 cases. Ann Oncol. 2007;18(12):2030–2036.
3. Kielhorn J, Melber C, Wahnschaffe U, et al. Vinyl chloride: still a cause for concern. Environ. Health Perspect. 2000;108(7):579–588.
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6. Kim HR, Rha SY, Cheon SH, et al. Clinical features and treatment outcomes of advanced stage primary hepatic angiosarcoma. Ann Oncol. 2009;20(4):780–787.
7. Peterson MS, Baron RL, Rankin SC. Hepatic angiosarcoma: findings on multiphasic contrast-enhanced helical CT do not mimic hepatic hemagioma. AJR Am J Roentgenol 2000;175(1):165-170.
8. Mitsui F, Aikata H, Hashimoto Y, et al. A first case of hepatic angiosarcoma treated with recombinant interleukin-2. Hiroshima J Med Sci. 2011;60(4):91–96.