Breast Angiosarcoma Metastatic

Breast angiosarcoma that has spread to other parts of the body is a rare and aggressive form of cancer that presents unique challenges for patients and doctors. Understanding this disease and available treatment options can help patients navigate their journey with greater confidence.

Table of contents

• What is breast angiosarcoma
• Types of breast angiosarcoma
• Understanding metastatic disease
• Symptoms and presentation
• How it is diagnosed
• Treatment approaches
• Disease outlook

What is breast angiosarcoma

Breast angiosarcoma is a very rare type of cancer that starts in the cells that line the blood vessels or lymph vessels in the breast. Angiosarcoma, which combines the Greek words for “vessel” (angios) and “flesh” (sarcoma), describes a cancer that originates from vessels and can spread to other organs.^[1]

This disease is extremely uncommon. For every 1 million people in the United States, only one will be diagnosed with an angiosarcoma each year. Because so few people are diagnosed with this disease, researchers are still trying to determine how best to stop it from spreading or coming back.^[1]

Angiosarcoma represents about 2% of all sarcomas and is classified as a highly aggressive malignant tumor. The disease can occur in any location of the body, but about 60% of cases affect the skin, particularly the head and neck area.^[7]

Types of breast angiosarcoma

There are two main types of breast angiosarcoma, which differ based on how they develop:^[1]

Primary breast angiosarcoma occurs in people who have never had breast cancer before. This type most commonly develops when people are in their 30s and 40s. The causes and risk factors of primary breast angiosarcoma are not as well understood as the secondary form, mainly because of how rare this cancer is.^[1]

Secondary breast angiosarcoma, also called treatment-related or radiation-induced breast angiosarcoma, occurs in people who have been previously treated for breast cancer. This type tends to develop later in life, typically 7 to 10 years after radiation treatment. In addition to radiation therapy, some research suggests that removal of lymph nodes and chronic lymphedema (swelling) in the breast or arm may increase the risk of breast angiosarcoma.^[1]

If you have received radiation therapy for breast cancer, your risk of developing breast angiosarcoma may be higher. A report from 2019 estimated that 1 in 1,000 people who had radiation for breast cancer went on to develop angiosarcoma in the breast or chest wall.^[1]

Understanding metastatic disease

When breast angiosarcoma becomes metastatic, it means the cancer has spread beyond the breast to other parts of the body. This is an advanced stage of the disease that requires different treatment approaches than cancer that remains in the breast.^[5]

Angiosarcoma is an easily infiltrative tumor with a high rate of local recurrence and spread to distant sites. The reported rates of advanced or metastatic disease at the time of diagnosis vary from 16 to 44%.^[7]

A case report described a 36-year-old woman with pregnancy-associated angiosarcoma who progressed rapidly while on treatment. Her disease showed multiple areas of spread, including liver involvement and pleural effusion (fluid around the lungs). This case illustrates the aggressive nature of metastatic breast angiosarcoma.^[5]

The most common sites where angiosarcoma spreads include the lungs, bone, soft tissue, and skin. A specific type called epithelioid angiosarcoma often presents with early spread to lymph nodes and solid organs, especially to the lungs.^[10]

Symptoms and presentation

The symptoms of breast angiosarcoma can vary depending on whether it is primary or secondary, and whether it has spread to other areas of the body.^[1]

Symptoms of primary breast angiosarcoma can include:^[1]

• A thickened area of skin on the breast
• A lump in the breast
• Discolored rash or bruised appearance on the skin of the breast or arm

Symptoms of secondary breast angiosarcoma can include:^[1]

• Swelling in the breast or arm
• A lump in the breast
• Discolored rash or bruised appearance on the skin of the breast or arm

While many people with primary angiosarcoma often present with a mass in the breast, secondary angiosarcoma can often appear as a skin tag, pimple, or purple or blue-hued lesion on the breast. This can lead to delays in diagnosis, as patients may initially seek treatment from a dermatologist thinking it is a skin problem.^[1]

When the disease has become metastatic, patients may experience additional symptoms depending on where the cancer has spread. In one documented case, a patient presented with progressive abdominal distension, swelling in both legs, jaundice (yellowing of the skin), and difficulty breathing.^[5]

How it is diagnosed

If you identify a breast lump, pimple, or a bruise on your breast that is not going away, you should contact your doctor right away. They will likely perform a series of tests to determine what is causing your symptoms.^[1]

The diagnostic process typically includes a physical examination, imaging studies, and tissue sampling. Ultrasound, CT scans, and MRI are important diagnostic tools. However, the final diagnosis requires examination of tissue under a microscope and special staining tests called immunohistochemistry.^[7]

For metastatic disease, additional imaging may be performed to determine the extent of spread. In documented cases, positron emission tomography computed tomography (PET CT) scans have been used to identify areas where the cancer has spread, such as to the liver, lungs, or other organs.^[5]

The cancer cells in angiosarcoma express properties similar to endothelial cells (the cells that line blood vessels), and this can be confirmed through special laboratory tests on the biopsy sample.^[10]

Treatment approaches

Treatment for metastatic breast angiosarcoma is challenging because the disease is so rare and aggressive. Various approaches have been tried, often with limited success.^[5]

Chemotherapy is a common treatment approach for metastatic disease. Several drugs have been used, with varying results:^[9]

• Paclitaxel: Angiosarcoma is highly sensitive to this drug, which belongs to a class called taxanes. It has proved well tolerated and active even in previously treated patients with locally advanced or metastatic angiosarcoma. It is often given weekly as a single agent.
• Anthracycline-based regimens: These include drugs like doxorubicin and epirubicin, often combined with other medications.
• Gemcitabine-based regimens: Gemcitabine can be combined with docetaxel, vinorelbine, or dacarbazine.

Response to chemotherapy is typically only 40-50% with the most active regimens, and toxicity can be significant.^[9]

Targeted therapies are being investigated. Some patients with angiosarcoma that has specific genetic changes in vascular signaling genes may respond well to drugs like pazopanib. In one study of patients previously treated with standard chemotherapy, some patients with angiosarcoma responded to pazopanib.^[9]

An interesting approach involves the combination of propranolol (a beta-blocker medication) with low-dose chemotherapy, called metronomic chemotherapy. One report described patients with advanced angiosarcoma treated with twice-daily propranolol combined with weekly low-dose vinblastine and methotrexate. All patients responded, with some showing very good responses. In 2017, propranolol was granted special drug status in Europe for the treatment of soft tissue sarcoma.^[9]

A documented case described a young woman with metastatic primary angiosarcoma who was treated with a metronomic combination of oral drugs due to her poor general condition. She achieved disease stabilization, although the response was not long-lasting.^[5]

Surgery and radiation therapy remain important for local disease control, but their role is limited once the cancer has spread to distant sites.^[9]

Disease outlook

The prognosis for metastatic breast angiosarcoma is generally poor. The disease follows an aggressive course, and managing relapsing metastatic disease presents significant challenges.^[5]

The overall survival for patients with advanced or metastatic angiosarcoma ranges from 6 to 16 months. The disease has a high rate of local recurrence and distant spread.^[7]

In one study of patients with advanced vascular sarcomas who were treated with pazopanib after previous chemotherapy, the median progression-free survival was 3 months and median overall survival was 9.9 months.^[9]

Primary angiosarcoma of the breast is described as a highly aggressive but rare malignant growth. Different chemotherapy agents and combinations have been tried in the metastatic setting with generally poor results.^[5]

While the outlook remains challenging, ongoing research into targeted therapies and new treatment combinations offers hope for improved outcomes in the future. Because of the rarity of this disease, patients may benefit from seeking care at specialized cancer centers and considering participation in clinical trials when available.