Liver Angiosarcoma
Disease Details
Family Health Simplified
- Description
- Liver angiosarcoma is a rare and aggressive cancer originating from the blood vessels of the liver.
- Type
- Liver angiosarcoma is a rare and aggressive malignant tumor of the liver that originates from the endothelial cells lining the blood vessels. It is typically not considered to have a hereditary genetic transmission. Instead, it is often associated with certain environmental risk factors such as exposure to vinyl chloride, thorium dioxide (Thorotrast), and arsenic.
- Signs And Symptoms
- Liver angiosarcoma usually presents with vague and non-specific symptoms such as abdominal pain, abdominal distension (which are the two most common symptoms, occurring in around 60% of individuals), weight loss, fatigue or abdominal masses and liver disease like symptoms such as fever, malaise, anorexia and vomiting.Paradoxically, liver function is generally maintained until the final stages of the disease, further complicating diagnosis.
- Prognosis
- Due to the aggressive nature and high recurrence rate, the prognosis for liver angiosarcoma is generally very poor. Most patients die within six months and only 3% live more than two years.A recent case report suggest that the prognosis of liver angiosarcoma may be improving.
- Onset
- Liver angiosarcoma is a rare and aggressive cancer of the blood vessels in the liver. The exact onset is not well-defined due to its rarity and the typically asymptomatic nature of early-stage disease. When symptoms do appear, they often include abdominal pain, weight loss, fatigue, jaundice, or a palpable mass in the abdomen. Generally, it is diagnosed in individuals around 60 to 70 years old. Risk factors include exposure to certain chemicals such as vinyl chloride, arsenic, and thorium dioxide, as well as a history of chronic liver disease.
- Prevalence
- Liver angiosarcoma is an extremely rare and aggressive cancer of the liver. Its prevalence is exceedingly low, typically accounting for about 1-2% of primary liver cancers.
- Epidemiology
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Although liver angiosarcoma can affect anyone, most people affected are 60–70 years old. Males are affected more often than women at a ratio of 3-4:1, although in children, girls are affected more commonly than boys.
== References == - Intractability
- Liver angiosarcoma is considered highly intractable. This rare and aggressive cancer typically has a poor prognosis due to its rapid progression, resistance to treatment, and the difficulty of early detection. Treatment options like surgery, chemotherapy, and radiation therapy generally provide limited success, and the disease often recurs.
- Disease Severity
- Liver angiosarcoma is a rare and aggressive cancer originating in the blood vessels of the liver. The disease severity is high, often characterized by rapid progression, early metastasis, and poor prognosis. Early detection is challenging, and treatment options are limited, which contributes to its high mortality rate.
- Healthcare Professionals
- Disease Ontology ID - DOID:268
- Pathophysiology
- Liver angiosarcoma is a rare and aggressive malignant tumor originating from the endothelial cells lining the blood vessels of the liver. The pathophysiology involves the transformation of these endothelial cells into cancerous cells, which then proliferate uncontrollably and form irregular vascular channels. This can lead to extensive bleeding, liver dysfunction, and metastasis. Exact causes often include exposure to certain risk factors such as vinyl chloride, thorium dioxide (Thorotrast), arsenic, and anabolic steroids, although some cases are idiopathic. The prognosis is generally poor due to its aggressive nature and late presentation.
- Carrier Status
- Liver angiosarcoma is not typically associated with a carrier status, as it is a rare and aggressive type of cancer that originates in the blood vessels of the liver. It is primarily linked to exposure to certain chemicals, such as vinyl chloride and arsenic, rather than genetic inheritance or carrier status.
- Mechanism
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Liver angiosarcoma is a rare and aggressive malignant tumor originating from the endothelial cells lining blood vessels in the liver. The exact mechanisms driving liver angiosarcoma are not fully understood, but certain risk factors and molecular mechanisms have been identified.
**Mechanism:**
- **Carcinogenic Exposure:** Chronic exposure to carcinogens such as vinyl chloride, thorium dioxide (Thorotrast), and arsenic is strongly associated with the development of liver angiosarcoma. These agents induce DNA damage and mutations in hepatic endothelial cells.
- **Chronic Hepatitis and Cirrhosis:** Chronic liver disease and cirrhosis may increase the risk by creating an environment conducive to tumor development.
**Molecular Mechanisms:**
- **Genetic Mutations:** Mutations in the TP53 gene, a tumor suppressor, are commonly observed, leading to loss of cell cycle control and apoptosis.
- **KDR/VEGFR2 Gene Amplification:** Amplification of the KDR/VEGFR2 gene, which encodes for vascular endothelial growth factor receptor 2 (VEGFR2), promotes angiogenesis and tumor growth.
- **PIK3CA and PTEN Mutations:** Alterations in the PI3K/AKT pathway, through mutations in PIK3CA or loss of PTEN, contribute to increased cell proliferation and survival.
- **Angiogenic Factors:** Overexpression of angiogenic factors like VEGF (vascular endothelial growth factor) and FGF (fibroblast growth factor) helps in sustaining the growth of the endothelial tumor cells.
- **Epigenetic Changes:** Alterations in DNA methylation and histone modification patterns can also play a role in the deregulation of gene expression involved in tumorigenesis.
These molecular alterations lead to uncontrolled proliferation, resistance to apoptosis, and increased angiogenesis, which are hallmarks of liver angiosarcoma. - Treatment
- Complete surgical resection combined with adjuvant chemotherapy is considered to be the most effective treatment of liver angiosarcoma.Transcathether arterial embolization (TAE), blocking an artery with the help of a catheter to prevent further bleeding or limit blood supply to the tumor, resulting in suppressed growth, is the most effective treatment for spontaneous rupture of the tumor resulting in intra-abdominal bleeding. Transcatheter arterial chemoembolization (TACE), which is the same as TAE, but also involves the regional injection of chemotherapy drugs, has shown effectiveness at increasing survival, particularly in individuals with few dominant masses rather than several smaller. TACE allows for simultaneously increasing regional (and therefore also tumor) exposure to chemotherapy while reducing systemic exposure, which both allows for an increased dose, as systemic exposure is generally the limiting factor, and also reduced side effects of chemotherapy. TACE and TAE are both generally performed on the hepatic artery.Liver angiosarcomas are generally reported to be radioresistant and therefore radiation therapy is not considered an effective treatment.Although previously considered a viable treatment option, liver transplantation is no longer considered for liver angiosarcoma, due to its high reoccurrence rate and poor post transplantation survival. The European Liver Transplant Registry considers liver angiosarcoma an absolute contraindication to liver transplantation, reporting that the median survival following liver transplantation is less than 7 months with no one surviving more than 23 months, showing very little difference from no treatment at all.
- Compassionate Use Treatment
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Liver angiosarcoma is a rare and aggressive cancer that originates in the blood vessels of the liver. Due to its rarity, standard treatments are limited, making compassionate use and off-label treatments of interest.
1. **Compassionate Use Treatments**:
- **Immunotherapy**: Medications like pembrolizumab (Keytruda) or nivolumab (Opdivo) may be available under compassionate use for patients who have exhausted standard treatment options.
- **Investigational Drugs**: Access to experimental medications that are in clinical trials but not yet approved.
2. **Off-Label or Experimental Treatments**:
- **Chemotherapy**: Agents such as paclitaxel, ifosfamide, and doxorubicin, though not specifically approved for liver angiosarcoma, are sometimes used based on their efficacy in similar sarcomas.
- **Targeted Therapy**: Drugs like sorafenib and bevacizumab, approved for other cancers but used off-label for angiosarcoma.
- **Clinical Trials**: Participation in clinical trials evaluating new therapies, such as novel kinase inhibitors or combination therapies.
Patients considering these options should consult with their healthcare provider to understand the potential benefits and risks. - Lifestyle Recommendations
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For liver angiosarcoma, here are some lifestyle recommendations:
1. **Avoid Alcohol**: Consumption of alcohol can further damage the liver, so it is advisable to abstain from drinking.
2. **Healthy Diet**: Maintain a balanced diet rich in fruits, vegetables, whole grains, and lean proteins to support overall health and liver function.
3. **Avoid Exposure to Toxins**: Limit exposure to potential carcinogens such as vinyl chloride and arsenic, which are linked to the development of liver angiosarcoma.
4. **Regular Check-ups**: Keep up with regular medical appointments for monitoring the condition and managing symptoms.
5. **Exercise**: Engage in moderate physical activity to enhance overall well-being, but consult with your healthcare provider before starting any exercise regimen.
6. **Quit Smoking**: If you smoke, seek help to quit, as smoking can exacerbate health issues.
7. **Hydration**: Drink plenty of water to help flush out toxins and support liver health.
8. **Rest**: Ensure you get adequate sleep to allow your body to repair and restore itself.
Always consult with a healthcare professional for personalized advice and treatment plans. - Medication
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Liver angiosarcoma is a rare and aggressive cancer of the liver. There is no specific medication that can cure it, but treatment options generally include:
1. **Chemotherapy**: Drugs such as doxorubicin and ifosfamide may be used to control tumor growth.
2. **Targeted Therapy**: Agents like bevacizumab that inhibit blood vessel formation in tumors.
3. **Immunotherapy**: Drugs like pembrolizumab may be considered in certain cases.
4. **Symptomatic Treatment**: Medications for pain management and other symptoms related to the disease.
Treatment plans are personalized and determined by the oncologist based on the patient's overall health and stage of the disease. - Repurposable Drugs
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Liver angiosarcoma is a rare and aggressive cancer originating in the blood vessels of the liver. Due to its rarity and aggressiveness, there is limited data on effective treatments. However, certain drugs have shown potential in repurposing efforts:
1. **Paclitaxel**: An established chemotherapy drug used in various cancers, including angiosarcomas outside the liver.
2. **Bevacizumab**: A monoclonal antibody targeting vascular endothelial growth factor (VEGF), which may inhibit tumor angiogenesis.
3. **Sorafenib**: A tyrosine kinase inhibitor used in liver cancer (hepatocellular carcinoma) that may have potential benefits.
These drugs are studied in contexts other than liver angiosarcoma and may require further clinical trials to confirm efficacy and safety. Always consult a specialist for personalized medical advice. - Metabolites
- Liver angiosarcoma, a rare and aggressive malignancy of the liver, does not have specific metabolites that are unique or diagnostic for the condition. Instead, liver function tests might show elevated liver enzymes, but these are nonspecific. Diagnostic approaches typically involve imaging studies and biopsy.
- Nutraceuticals
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Liver angiosarcoma is a rare and aggressive cancer originating in the blood vessels of the liver. There is no specific evidence supporting the efficacy of nutraceuticals (dietary supplements with potential health benefits) in treating liver angiosarcoma. Standard treatment typically involves surgery, chemotherapy, and radiation.
"NAN" could refer to "nanotechnology," which is an emerging field being explored for potential applications in cancer treatment, including drug delivery systems targeting tumor cells more precisely. However, research in this area is still in the experimental stages and not yet a standard treatment for liver angiosarcoma. Consulting a healthcare professional for current and personalized treatment options is recommended. - Peptides
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In the context of liver angiosarcoma:
**Peptides:** These are short chains of amino acids and can play a role in targeted therapies, although their specific application in liver angiosarcoma is still under research. Peptide-based drugs or therapies may help in targeting tumor-specific molecules to minimize damage to healthy cells.
**Nanotechnology (Nan):** Nanotechnology offers potential in diagnosing and treating liver angiosarcoma by delivering drugs directly to cancer cells, improving imaging techniques, and reducing side effects of conventional treatments. Nanoparticles can be engineered to carry chemotherapeutic agents, enhance imaging contrast, or deliver genetic material to cancer cells.