Angiosarcoma of The Liver

Cancer is a scary and deadly disease. In the UK, there are around 167,000 cancer-related deaths every year, meaning an estimated 460 deaths every day.1 Among all the cancers, the statistics for liver cancer are particularly harrowing when you consider it is the 3rd highest cause of cancer-related deaths in the world.2This article, therefore, focuses on liver cancer and its types and pays special attention to one particular type of liver cancer: Liver Angiosarcoma.

Types of Liver Cancer

There are two broad categories of liver cancer:

  1. Primary Liver Cancer 
  2. Secondary Liver Cancer

As the name suggests, primary liver cancer refers to cancer that has started in the liver

There are five types of primary liver cancer:3

  1. Hepatocellular Carcinoma: This is the most common type of liver cancer, and stems from issues within the hepatocytes [hepatocytes are primary cells of the main functional tissue of the liver, comprising the majority of its mass]. This type of cancer usually occurs due to cirrhosis (scarring of the liver tissue) which usually happens due to infections such as Hepatitis B or C or due to long-term alcohol consumption. The disease is more prevalent in the elderly and men compared to women.
  2. Fibrolamellar Carcinoma: A rather rare subtype of liver cancer, this occurs in individuals with healthy livers and usually the patients are aged between 20 and 30. 
  3. Bile duct cancer (also known as Cholangiocarcinoma): The phrase cholangitis is a reference to the bile ducts [bile ducts are thin tubular-like structures that allow a fluid known as bile to run from the liver to the small intestine. Bile is a yellowish fluid that aids in the digestion and metabolism of fats]. So this cancer is a cancer of the bile ducts and is considered to be quite rare.
  4. Angiosarcoma: The highlight of this article, this cancer is also known as hemangiosarcoma and begins in the blood vessels of the liver. It is often found in the elderly.
  5. Hepatoblastoma: Considered to be an extremely rare type of cancer, this is often found in children under the age of 2. As the name suggests, cancer originates from the liver cells.

What is Liver Angiosarcoma?

Now that we have understood the types of liver cancers let’s take a deep dive into what Liver or Hepatic Angiosarcoma is. But before that we need to understand the term “Angiosarcoma” - Angiosarcoma occurs when there is a malignant growth of tissues that results from an abnormal division and growth of cells or lack of apoptosis (normal and controlled cell death).

Types of Angiosarcoma

Angiosarcoma most commonly occurs in the head, neck, and breast but the liver is the fifth most common place for cancer. Liver angiosarcoma is considered to be very aggressive.4

How Common is Liver Angiosarcoma?

Liver or Hepatic Angiosarcoma is the third most common type of primary liver cancer and accounts for approximately 0.1% to 2% of all primary liver cancers. That said, it is a very rare disease accounting for 200 cases worldwide annually. It usually affects men more than women and especially elderly men. However, if it were to occur in childhood, there would be a greater chance of it occurring in girls compared to boys.5 

Signs and Symptoms

One of the things that makes this difficult to diagnose is the lack of specific symptoms. Patients usually present with pain in the upper right abdominal area, abdominal swelling, weight loss, jaundice, and fatigue. On physical examination, physicians may also find an enlarged liver (known as hepatomegaly) or fluid build-up in the abdomen (known as ascites). Patients may also present without any symptoms and the issue may only be found upon imaging.4

Causes and Risk Factors

Angiosarcoma in general does not have any specific causes. However, exposure to industrial materials such as arsenic, radium, and thorium dioxide, steroid use, and the use of oral contraceptives could lead to tumor formation. It has been speculated that Liver angiosarcoma in specific could arise from environmental influences.6

Diagnosis

Due to the lack of very specific symptoms, it is difficult to diagnose the disease. Most commonly, it is found on incidental abdominal imaging. The best forms of imaging for getting an accurate diagnosis would be a computed tomography scan (CT scan) followed by MRI. Lab studies are another crucial component for diagnosis. Normally, an affected individual will have elevated levels of enzymes such as alanine transaminase (the enzyme is responsible for cellular energy production) and aspartate transaminase (this enzyme deals with amino acid metabolism). While making a diagnosis for the disease it is pertinent that both the radiological and imaging results are scrutinized with equal importance.4

Treatments

for the disease are generally surgical and involve either hepatic resection or radial resection. Hepatic or liver resection is the removal of a piece of the liver, and this can constitute up to one-half of the liver. The procedure entails making an incision in the belly to remove the affected section. In case the tumor is on the right side of the liver and the surgery needs to be done on the right, the physician will also remove the gallbladder. After the surgery, the patient may have nausea, constipation, gas, headache, and/ or low fever. Furthermore, the surgical area will be sore for a few weeks, and it would take four to eight weeks for complete recovery.7 However, if cancer has metastasized these treatments would not be recommended. A liver transplant is also not an option, due to relatively high chances of recurrence and finally, no effective chemotherapy treatment has also been found yet.

Outlook/Prognosis

The prognosis for patients with liver angiosarcoma is quite grim. Most die within six months of diagnosis and even after treatment, the majority live for around 2 years. Including the delayed diagnosis, the average 5-year survival rate is approximately 35%.4

Prevention

While there are no defined ways to reduce the risk of liver angiosarcoma specifically, there are steps you can take - for example, to stay away from risk factors such as industrial chemicals - to remain safe. In addition, the ideal thing to do would be to stay active, eat healthily, prevent excessive or alcohol consumption (if possible), and also to stop smoking. These are common knowledge yet are crucial to prevent getting cancer or for that matter any serious or life-threatening diseases.4 

Current Research and Clinical Trials

A few studies have recently shown that using chemotherapy with chemicals such as Doxorubicin and ifosfamide could be helpful. Furthermore, research has shown that operative treatments usually lead to better outcomes in patients with stage 1 liver angiosarcoma when compared to non-operative treatments.4

Conclusion

To briefly outline the key points of the article, Liver angiosarcoma is a particularly rare disease with nonspecific symptoms and hence is difficult to diagnose. It also has a poor prognosis/outlook and a high mortality rate. Treatments are predominantly surgical. If you have any symptoms that might be related to cancer such as weight loss or severe fatigue it is best to get yourself checked out by your physician. 

References:

  1. Cancer Research UK. Cancer Statistics for the UK [Internet]. Cancer Research UK. CRUK; 2018. Available from: https://www.cancerresearchuk.org/health-professional/cancer-statistics-for-the-uk
  2. Liver Cancer - Statistics [Internet]. Cancer.net. 2019. Available from: https://www.cancer.net/cancer-types/liver-cancer/statistics
  3. Types | Liver cancer | Cancer Research UK [Internet]. www.cancerresearchuk.org. Available from: https://www.cancerresearchuk.org/about-cancer/liver-cancer/types
  4. Kumar A, Sharma B, Samant H. Liver Angiosarcoma [Internet]. PubMed. Treasure Island (FL): StatPearls Publishing; 2022 [cited 2022 Sep 26]. Available from: https://www.ncbi.nlm.nih.gov/books/NBK538224/
  5. Marks EI, Pamarthy S, Dizon D, Birnbaum A, Yakirevich E, Safran H, et al. ROS1-GOPC/FIG : a novel gene fusion in hepatic angiosarcoma. Oncotarget [Internet]. 2019 Jan 4 [cited 2022 Sep 26];10(2):245–51. Available from: https://www.oncotarget.com/article/26521/text/
  6. Hospital S. What Causes Angiosarcoma of The Liver? [Internet]. Sahyadri Hospital. 2022 [cited 2022 Sep 26]. Available from: https://sahyadrihospital.com/blog/what-causes-angiosarcoma-of-the-liver/
  7. Liver Resection: What to Expect at Home [Internet]. myhealth.alberta.ca. [cited 2022 Sep 26]. Available from: https://myhealth.alberta.ca/Health/aftercareinformation/pages/conditions.aspx?hwid=zc2226#:~:text=Liver%20resection%20is%20surgery%20to
This content is purely informational and isn’t medical guidance. It shouldn’t replace professional medical counsel. Always consult your physician regarding treatment risks and benefits. See our editorial standards for more details.

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Punyaslok Mishra Mishra

MB BCh BAO - Queen's University Belfast, Northern Ireland

Punyaslok is an emerging medical professional from Queen's University Belfast with a specialization in Medicine. He has showcased leadership as the President of the Asian Medical Students’ Association in Northern Ireland since August 2022. Besides, he contributes as a Peer Mentor and has recently undertaken a vital role as a Medical Writer Intern at Klarity, where he pens insightful articles for a health library, discussing topics from angina to the enzymes in papaya. Notably, Punyaslok's research on the potential of Mesenchymal Stem Cells in treating Anthracycline Induced Cardiomyopathy is affiliated with Queen's University, signifying his deep interest in advancing therapeutic measures in the medical realm.

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