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]
- Epigastric Mass
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
- 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]
Microscopically, the liver mass revealed a high-grade malignant neoplasm composed of epithelioid cells exhibiting marked cellular pleomorphism, brisk mitotic activity ( 50 mitotic figures/10 high power fields) and extensive geographic tumor necrosis [pagepress.org]
Radiation: along with chemotherapy this may relieve the pain of large liver masses, and radiation to painful bone or other metastases may be also appropriate Investigational methods: combination chemo or new drugs including derivatives of Adriamycin and [cancersupportivecare.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.
- 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]
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]
Liver biopsy, either percutaneous or transjugular, is often required in patients with reasonable performance status. [karger.com]
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]
Even with an established diagnosis, treatment of the tumor has yet to be well defined and is largely empirical. [karger.com]
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]
Etiology Most 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]
126 (75%) were attributed to uncertain etiology [ 2 ]. [bmcgastroenterol.biomedcentral.com]
Publication of this report and a related journal article (5) stimulated a series of clinical investigations, epidemiologic studies, and toxicologic analyses. [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]
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]
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]
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]
Prevention - Angiosarcoma of the liver Not supplied. Diagnosis - Angiosarcoma of the liver Not supplied. [checkorphan.org]
A SHE has been defined as "an unnecessary disease, disability, or untimely death whose occurrence signals a failure of prevention" (3). [cdc.gov]
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]
Google Scholar Chen D-S: Hepatitis B virus infection, its sequelae, and prevention in Taiwan. Neoplasms of the Liver. Edited by: Okuda K, Ishak KG. 1987, Tokyo: Springer-Verlag, 71-80. [bmcgastroenterol.biomedcentral.com]
There is no definitive method to prevent Primary Cardiac Angiosarcoma occurrence. [dovemed.com]
- Forbes A, Portmann B, Johnson P, Williams R. Hepatic sarcomas in adults: a review of 25 cases. Gut. 1987;28(6):668–674.
- 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.
- Kielhorn J, Melber C, Wahnschaffe U, et al. Vinyl chloride: still a cause for concern. Environ. Health Perspect. 2000;108(7):579–588.
- Lee SW, Song CY, Gi YH, et al. Hepatic angiosarcoma manifested as recurrent hemoperitoneum. World J Gastroenterol. 2008;14(18):2935–2938.
- Leowardi C, Hormann Y, Hinz U, et al. Ruptured angiosarcoma of the liver treated by emergency catheter-directed embolization. World J Gastroenterol. 2006;12:804–808.
- 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.
- 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.
- 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.