Angiosarcoma Metastatic

Metastatic angiosarcoma is an advanced and extremely aggressive form of a rare cancer that originates from blood or lymph vessel cells and has spread to other parts of the body, with survival measured in months rather than years.

Table of contents

• What Is Metastatic Angiosarcoma
• Where Angiosarcoma Spreads
• Survival and Prognosis
• Diagnosis
• Treatment Options

What Is Metastatic Angiosarcoma

Metastatic angiosarcoma is a rare and highly dangerous cancer that develops when angiosarcoma, a cancer that starts in the cells lining blood vessels or lymph vessels, spreads to distant parts of the body^[1]. Angiosarcoma itself represents only 1 to 2 percent of all soft tissue cancers in humans^[1]. When this cancer spreads beyond its original location to other organs, it becomes metastatic disease.

The cancer cells that line blood vessels and lymph vessels become abnormal and multiply uncontrollably, forming new irregular blood vessels that eventually develop into tumors^[5]. These cancerous cells continue growing and spreading from the affected blood vessels to other areas of the body^[6].

Between 16 and 44 percent of patients with angiosarcoma present with advanced or metastatic disease at the time of diagnosis^[1][8]. The disease is known for its aggressive behavior, high rate of spreading to distant sites, and poor response to treatment^[1].

Where Angiosarcoma Spreads

When angiosarcoma becomes metastatic, it typically spreads to specific organs in the body. Understanding these patterns helps doctors monitor the disease and plan treatment.

The most common sites where metastatic angiosarcoma spreads include the lungs, liver, bones, and brain^[4]. In one study examining patients with metastatic angiosarcoma, researchers found that 60 percent had spread to the lungs, making this the most frequent site^[4]. About 30 percent had disease in the liver, 43 percent in the bones, and 9 percent in the brain^[4].

A distinctive feature of metastatic angiosarcoma in the lungs is the appearance of cystic lung nodules with surrounding ground-glass changes on imaging scans^[8]. This characteristic pattern can help doctors identify the disease.

Many patients with metastatic angiosarcoma have cancer in more than one distant location. About 34 percent of patients in one study had two or more metastatic sites at the time of diagnosis^[4].

Survival and Prognosis

The outlook for people with metastatic angiosarcoma is extremely poor, with survival measured in months rather than years.

Studies show that the median overall survival for patients with metastatic angiosarcoma ranges from 6 to 16 months^[1]. In one detailed study of 284 patients with metastatic disease, the survival rates at one year and three years were only 21 percent and 4 percent, respectively^[4]. When looking specifically at deaths caused by the cancer itself, the cancer-specific survival rates were 22 percent at one year and just 5 percent at three years^[4].

This stands in stark contrast to patients whose angiosarcoma has not spread, who have a median survival of 20 months^[4]. Some research indicates that patients with localized disease may have five-year survival rates ranging from 10 to 50 percent^[4], though outcomes vary widely.

Several factors influence how long a person with metastatic angiosarcoma may survive. Tumors larger than 10 centimeters are associated with worse outcomes^[4]. Higher tumor grades, which indicate more aggressive cancer cells, also predict poorer survival^[4]. Age appears to play a role as well, with older patients generally having shorter survival times^[4].

Diagnosis

Diagnosing metastatic angiosarcoma requires confirming both the presence of angiosarcoma and identifying where it has spread in the body.

The definitive diagnosis of angiosarcoma requires taking a small sample of tissue, called a biopsy, which is then examined under a microscope^[1][5]. Laboratory specialists use special staining techniques called immunohistochemistry to confirm the diagnosis and distinguish angiosarcoma from other types of cancer^[1].

To determine whether the cancer has spread to distant organs, doctors use several imaging tests. CT scans (computed tomography) play an important role in evaluating metastatic disease^[8]. These scans can detect tumors in the lungs, liver, bones, and other organs. MRI (magnetic resonance imaging) is preferred for assessing the primary tumor site and planning treatment^[8]. Other imaging techniques such as ultrasound may also be used depending on the location being examined^[1].

Additional tests may include PET scans to identify areas of cancer activity throughout the body^[5]. Blood tests, while not diagnostic on their own, may be performed to assess overall health and organ function^[1].

Treatment Options

Treatment for metastatic angiosarcoma focuses on slowing cancer growth and managing symptoms, as curing the disease at this advanced stage is rarely possible. The approach typically involves chemotherapy, sometimes combined with other treatments.

Chemotherapy is the main treatment recommended for metastatic angiosarcoma^[4]. Studies have shown that chemotherapy is an independent favorable predictor of survival in patients with metastatic disease^[4]. Research demonstrates that chemotherapy is the preferred treatment option for these patients^[4].

Several chemotherapy drugs have shown activity against angiosarcoma. Paclitaxel, a drug in the taxane family, is highly effective and well tolerated even in patients who have received prior treatment^[11]. It is typically given weekly^[11]. Doxorubicin, an anthracycline drug, is another recommended option for advanced or metastatic disease^[6][11]. Combinations of gemcitabine with other drugs such as docetaxel or vinorelbine may also be used^[11].

Radiation therapy may provide benefit in metastatic angiosarcoma, particularly when combined with chemotherapy. Studies indicate that radiation treatment is an independent favorable predictor of overall survival^[4]. Radiation can be used to treat specific areas where the cancer has spread, helping to control symptoms and slow disease progression^[11].

Targeted therapy represents a newer approach that attacks specific features of cancer cells. Because angiosarcomas develop from blood vessel cells, drugs that target blood vessel growth factors have been studied. Pazopanib, which blocks vascular endothelial growth factor receptors, has shown responses in some patients with advanced angiosarcoma^[11]. In one study, eight out of 40 patients with angiosarcoma responded to pazopanib, with median survival of almost 10 months^[11].

Immunotherapy, which harnesses the immune system to fight cancer, represents an exciting development in angiosarcoma treatment. Drugs targeting the PD-1 receptor or PD-L1 protein have been explored^[13]. Case reports describe patients with metastatic angiosarcoma achieving marked shrinkage of tumors and prolonged responses with immunotherapy drugs like pembrolizumab^[13]. However, these treatments are still being studied and are not yet standard therapy.

Surgery has a limited role in metastatic angiosarcoma. One study found that surgery did not improve survival in patients with metastatic disease^[4]. Surgery may occasionally be considered for removing isolated metastases, but this is uncommon.

The best outcomes often result from a multidisciplinary approach, where cancer specialists from different fields work together to create an individualized treatment plan^[1][6]. This team may include medical oncologists, radiation oncologists, surgeons, and other healthcare professionals.