Angiosarcoma of the liver is a rare cancer that is rapidly progressive and carries a poor outcome. 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.
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 . 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 . Symptoms such as general malaise, significant loss of weight and tiredness have been reported . In contrast to the latter, another common presentation is a localized abdominal mass or generalized abdominal distension, in an otherwise clinically healthy patient . 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 . 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  .
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 .
Entire Body System
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]
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]
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]
He endorsed decreased appetite, fatigue, and minimal weight loss during the preceding two months, but otherwise had previously felt healthy. [cureus.com]
The patient usually presents with nonspecific symptoms such as fatigue, weight loss and abdominal discomfort, followed by ascites, icterus and hepatomegaly. In the present case, however, the patient presented with hematemesis and melena. [karger.com]
Case 1 presented in August 1967 with an epigastric mass and thrombocytopenia. An exploratory laparotomy was performed in September 1967; liver biopsy revealed angiosarcoma. Case 2 presented in January 1970 with gastrointestinal (GI) bleeding. [cdc.gov]
mass DM, HTN, liver rupture No none TAE OP none 4 M CD31 7 F 60 liver tumor hypothyroidism, uremia No HCV C none 2 M CD34 8 F 63 right abdominal pain liver rupture No none OP C/T peritoneum, duodeum, colon 3 M CD31 9 M 56 liver tumor L/C, EV, left hip [bmcgastroenterol.biomedcentral.com]
Liver, Gall & Pancreas
Hepatic mass and adjacent liver parenchyma with associated capsular disruption and inflammatory reaction (H&E stain, 40x magnification). [pagepress.org]
Proliferation of hepatic masses after the first year is less characteristic of IHE and should raise suspicion of a malignant tumor. [cancerjournal.net]
HA can present as an inconclusive multifocal or focal hepatic mass with broad differential diagnoses such as liver abscess, hepatocellular cancer, hepatic metastasis, primary hepatic lymphoma, hepatic cystadenocarcinoma, hemangioblastoma and hepatic epithelioid [karger.com]
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 . 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 . 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.
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]
An exploratory laparotomy was performed in September 1967; liver biopsy revealed angiosarcoma. Case 2 presented in January 1970 with gastrointestinal (GI) bleeding. [cdc.gov]
Conclusions Liver biopsies are difficult to obtain from patients with cryptogenic liver failure. [surgicalcasereports.springeropen.com]
An open liver biopsy done at this time showed features of a vascular tumor as described above. Also seen were cavernous spaces at the edge of the tumor [Figure 4] a. [cancerjournal.net]
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