Liver Angiosarcoma

1. Disease Summary:

Liver angiosarcoma is a rare and aggressive malignant tumor that originates from the endothelial cells of blood vessels in the liver. It is classified as a type of soft tissue sarcoma and is characterized by rapid growth and a poor prognosis. The tumor is often diagnosed at an advanced stage due to nonspecific symptoms, which may include abdominal pain, weight loss, and jaundice. The rarity of the disease, combined with its aggressive nature, makes it a challenging condition to manage.

2. Global Prevalence and Disease Burden:

Liver angiosarcoma accounts for approximately 1-2% of all primary liver tumors and is more common in adults, particularly those with a history of exposure to certain chemicals (e.g., vinyl chloride) or pre-existing liver conditions. The incidence is estimated to be around 0.1 to 0.5 cases per million people annually. Due to its rarity, comprehensive epidemiological data is limited, but the overall burden of liver cancer, including angiosarcoma, is significant, with liver cancer being the sixth most common cancer worldwide and the fourth leading cause of cancer-related deaths. The economic impact includes high healthcare costs associated with diagnosis, treatment, and palliative care, as well as loss of productivity due to illness.

3. Unmet Medical Need:

The unmet medical needs for liver angiosarcoma are substantial and multifaceted:

Lack of Effective Treatments: Current treatment options yield limited survival benefits. Surgical resection is the only potentially curative approach, but many patients present with advanced disease and are not surgical candidates. Chemotherapy and radiation therapy have shown minimal effectiveness, and there are no standardized treatment protocols specifically for liver angiosarcoma (Li et al., 2018; PMID: 29724676).
Poor Prognosis: The median overall survival for patients with liver angiosarcoma is typically less than one year, with many studies reporting median survival times ranging from 6 to 15 months (Ramakrishnan et al., 2023; PMID: 37580871). This highlights the urgent need for more effective systemic therapies.
Limited Research and Clinical Trials: There is a scarcity of clinical trials specifically targeting liver angiosarcoma, which hampers the development of new treatment options. Most existing studies focus on broader categories of liver cancer, leaving angiosarcoma under-researched (Qiu et al., 2025; PMID: 40083292).
Patient Quality of Life: The aggressive nature of the disease and the side effects of current treatments significantly impact patients' quality of life. There is a need for supportive care strategies that address the physical and emotional challenges faced by patients (Angiosarcoma Awareness Inc).

4. Current Treatment Options:

Current treatment options for liver angiosarcoma include:

Surgical Resection: This is the primary treatment for localized disease. However, only a minority of patients are eligible for surgery due to the advanced stage of the disease at diagnosis (Ramakrishnan et al., 2023; PMID: 37580871).
Chemotherapy: Various chemotherapeutic agents have been used, but the response rates are generally low. Common regimens include doxorubicin and ifosfamide, but these treatments often result in disease stabilization rather than curative outcomes (Li et al., 2018; PMID: 29724676).
Radiation Therapy: This is typically used for palliative care to relieve symptoms but is not curative (Martins et al., 2020; PMID: 33263653).
Liver Transplantation: This is considered in select cases, but the high recurrence rate of angiosarcoma post-transplant limits its applicability (Ramakrishnan et al., 2023; PMID: 37580871).

5. Current Clinical Trials:

There are limited clinical trials specifically targeting liver angiosarcoma. Most ongoing studies focus on broader categories of liver cancer or investigate novel therapies that may not yet have been validated for angiosarcoma. For instance, trials exploring immune checkpoint inhibitors and targeted therapies are emerging, but results specific to liver angiosarcoma are still pending (Qiu et al., 2025; PMID: 40083292).

6. Additional Context:

Patient advocacy groups, such as Angiosarcoma Awareness Inc, play a crucial role in raising awareness and funding research for angiosarcoma. They provide resources for patients and families, emphasizing the need for more research and better treatment options. Collaborative efforts between researchers, clinicians, and advocacy organizations are essential to address the unmet needs in liver angiosarcoma and improve patient outcomes.