Angiosarcoma

Angiosarcoma, a subtype of soft tissue sarcoma, is an aggressive cancer that develops in the lining of blood vessels or lymphatic vessels. It can occur anywhere in the body but is most common in the skin (especially the scalp), breast (often after radiation), or liver. It's challenging to treat and has a tendency to spread. 

It accounts for less than 1% of all soft tissue sarcomas. Estimates suggest that there are only a few hundred cases diagnosed in the United States each year. Given the rarity of this cancer, experienced care — like that found at AHN — can make a difference in your treatment options.

AHN hematologists, surgeons, and health care professionals have vast experience in diagnosing and treating angiosarcoma. At AHN, we see you and your unique needs, creating a personalized care plan for you. We use advanced treatment options with targeted precision.

AHN Sarcoma Center of Excellence

The AHN Sarcoma Center of Excellence is made up of a team of multiple health care experts walking you through every step of your cancer diagnosis and treatment.

Diagnosis and treatment will usually begin with an appointment with our orthopaedic oncology team. The team works closely with providers amongst different medical specialties including:

• Surgeons
• Medical Oncology
• Radiation Oncology
• Pathology
• Physical therapy
• Occupational therapy
• Plastic surgery
• Nurse Navigators

At AHN, we strive to make your treatment course as painless as possible. We will take care of appointment scheduling, imaging planning, and other aspects of care coordination for our patients so you have time to focus on healing and recovery.

Quick guide to angiosarcoma

Angiosarcoma symptoms and signs

Angiosarcoma screening and diagnosis

Types and stages of angiosarcoma

Angiosarcoma treatment

Angiosarcoma FAQs

Contact us 

Angiosarcoma symptoms and signs

The signs and symptoms of angiosarcoma may be hard to spot early on in the disease. They also can be mistaken for other conditions. It’s important to talk with your doctor about any new or worsening symptoms or health issues you are experiencing. With angiosarcoma, symptoms can vary greatly depending on the location of the tumor. Angiosarcoma is found in the following areas of the body and have the following symptoms and signs:

Skin angiosarcoma:

• Bruise-like or purple lesions or patches that don't go away
• Swelling, particularly in the affected area
• Lesions that may bleed easily
• Rapid growth of the skin lesion

Breast angiosarcoma:

• A new lump or mass in the breast
• Skin changes, such as discoloration, swelling, or redness
• Rapid growth of the mass in the breast tissue

Hepatic (relating to the liver) angiosarcoma:

• Abdominal pain or swelling
• Jaundice (yellowing of the skin and eyes)
• Fatigue
• Weight loss

Deep tissue angiosarcoma:

• A lump or mass that may or may not be painful
• Swelling
• Pain in the affected area

Causes and risk factors

While the exact cause of angiosarcoma is often unknown, several risk factors have been identified that can increase a person's likelihood of developing this rare cancer. Here's a breakdown of established risk factors for developing angiosarcoma:

• Radiation therapy: Prior radiation therapy, particularly for breast cancer, is a significant risk factor.
• Lymphedema: Chronic lymphedema (swelling caused by a blockage in the lymphatic system) is associated with angiosarcoma, especially in the arm after breast cancer surgery (Stewart-Treves syndrome).
• Chemical exposure: Exposure to certain chemicals, such as vinyl chloride (used in the production of PVC plastics) and arsenic, has been linked to liver angiosarcoma.
• Genetic conditions: In very rare cases, angiosarcoma may be associated with certain genetic conditions, although the specific genes involved are not always clear.

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Angiosarcoma screening and diagnosis

Since angiosarcoma is a rare cancer type, there is no standard screening protocol for the general population. Screening is generally not effective for rare cancers, as the risk of false positives and unnecessary testing outweighs the benefits. Instead, awareness of risk factors and symptoms is key.

The first exam is usually a medical exam with your doctor. You will be asked about your medical history, including any risk factors for angiosarcoma (e.g., previous radiation therapy, lymphedema, chemical exposure). A thorough physical examination will be performed to assess the affected area and look for any signs of angiosarcoma, such as skin lesions, masses, or swelling.

Imaging tests

The specific imaging modalities used will depend on the suspected location of the angiosarcoma, the stage of the disease, and the individual patient's circumstances. The following imaging tests may be used to detect if there are signs of angiosarcoma:

• X-rays: May be used to evaluate bone involvement or lung metastases.
• Ultrasound: Can be helpful for evaluating superficial lesions or masses.
• CT scan (Computed Tomography): Provides detailed images of internal organs and tissues and can help assess the size, location, and extent of the tumor. CT scans are often used to evaluate angiosarcomas in the liver, spleen, or other internal organs.
• MRI (Magnetic Resonance Imaging): Provides excellent soft tissue detail and is often used to evaluate angiosarcomas in the skin, soft tissues, or heart.
• PET Scan (Positron Emission Tomography): Can help detect areas of increased metabolic activity, which may indicate the presence of cancer. PET scans are often used to evaluate the spread of angiosarcoma to other parts of the body.
• Echocardiogram: Essential for cardiac angiosarcoma to visualize the tumor and assess heart function.

Biopsy

The only way to definitively diagnose angiosarcoma is through a biopsy. A tissue sample is taken from the suspicious area and examined under a microscope by a pathologist. Types of biopsies include:

• Incisional biopsy: A small piece of tissue is removed.
• Excisional biopsy: The entire suspicious area is removed.
• Core needle biopsy: A needle is used to extract a core of tissue.

The biopsy should be performed by an experienced AHN surgeon or interventional radiologist to ensure that an adequate sample is obtained and to minimize the risk of complications. A pathologist examines the sample to look for characteristic features of angiosarcoma, such as:

• Irregularly shaped blood vessels
• Abnormal endothelial cells (cells lining the blood vessels)
• Increased number of cell divisions (mitoses)
• Necrosis (tissue death)

Immunohistochemistry

Immunohistochemistry is a special test that is performed on the biopsy sample to identify specific proteins that are present in the cells. This can help confirm the diagnosis of angiosarcoma and distinguish it from other types of tumors. Common immunohistochemical markers used to diagnose angiosarcoma include:

• CD31
• CD34
• Factor VIII-related antigen
• ERG

Further staging

If angiosarcoma is diagnosed, further staging tests may be performed to determine the extent of the cancer and whether it has spread to other parts of the body.Staging tests may include:

• CT scans of the chest, abdomen, and pelvis
• MRI of the brain
• Bone scan
• PET scan

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Types and stages of angiosarcoma

Angiosarcoma is a rare type of cancer that arises from the cells that line the blood vessels and lymphatic vessels (endothelial cells). Because blood vessels are all over the body, angiosarcomas can occur almost anywhere, but certain locations are more common. The type of angiosarcoma often depends on where it originates.

Cardiac angiosarcoma

Cardiac angiosarcoma is a malignant tumor that arises from the endothelial cells lining the blood vessels of the heart. It's not a result of cancer spreading (metastasizing) from another part of the body. It's the most common type of primary cardiac sarcoma (cancer that originates in the heart), but even then, it's still incredibly rare. Diagnosis can be challenging because the symptoms can mimic other heart conditions, and the rarity of the disease means it's often not the first thing doctors suspect.

Because angiosarcomas originate from blood vessels, they can potentially occur in any part of the heart. Cardiac angiosarcomas most commonly arise in the right atrium (the upper right chamber of the heart). This is thought to be because the right atrium is larger and has a more complex structure than other heart chambers. Less frequently, they can occur in the left atrium, ventricles (lower chambers), or pericardium (the sac surrounding the heart). Cardiac angiosarcomas are known for being highly aggressive. They tend to grow rapidly and spread (metastasize) early to other parts of the body, such as the lungs, liver, and brain.

By microscopic appearance (histologic subtypes)

What angiosarcoma looks like under a microscope can vary depending on the grade of the tumor, the location, and other factors.

Epithelioid angiosarcoma (EAS)

EAS is a rare and aggressive subtype of angiosarcoma. What distinguishes it is its microscopic appearance. Under the microscope, the cancer cells (tumor cells) have an "epithelioid" morphology, which means they resemble epithelial cells (the cells that line surfaces in the body) more than the typical spindle-shaped cells seen in other angiosarcomas.

Other types of angiosarcoma that may be diagnosed under a microscope:

• Spindle cell angiosarcoma: Characterized by spindle-shaped tumor cells.
• Pleomorphic angiosarcoma: Characterized by a variety of cell shapes and sizes.
• Well-differentiated angiosarcoma: Shows more organized vascular structures and is often less aggressive.
• Poorly differentiated angiosarcoma: Shows disorganized vascular structures and is more aggressive.

Cutaneous angiosarcoma (skin angiosarcoma)

Cutaneous angiosarcoma is a rare and aggressive cancer that starts in the cells lining the blood vessels of the skin. It often appears as a bruise-like spot or reddish/purple nodule that doesn't heal, mainly on the scalp or face of older adults. Early diagnosis and treatment are crucial due to its aggressive nature.

• Head and neck angiosarcoma (Wilson-Jones Angiosarcoma): This is the most common type of angiosarcoma overall. It typically affects older adults (often over 70), and is often found on the scalp and face, especially in sun-damaged skin. It often presents as a bruise-like lesion that doesn't heal, or as a raised, reddish-purple nodule. It can sometimes be mistaken for a benign skin condition initially.
• Radiation-associated angiosarcoma: This type develops in areas that have previously been treated with radiation therapy, often for breast cancer, lymphoma, or other cancers. It can occur several years (often five to 10 years or more) after radiation exposure. It's important to note that while radiation is a risk factor, the absolute risk of developing this is still relatively low.
• Lymphedema-associated angiosarcoma (Stewart-Treves Syndrome): This is a rare type that occurs in patients with chronic lymphedema (swelling caused by a blockage in the lymphatic system). It's most often seen in women who have had a mastectomy with axillary lymph node dissection for breast cancer, leading to lymphedema in the arm.

Metastatic angiosarcoma

Metastatic angiosarcoma is a rare and aggressive type of cancer that begins in the lining of blood vessels or lymphatic vessels and has spread to other parts of the body. When it is metastatic, this means the cancer cells have spread (metastasized) from its original location to other parts of the body. Cancer cells can break away from the primary tumor and travel through the bloodstream or lymphatic system to form new tumors in distant organs or tissues.

Soft tissue angiosarcoma

This type arises in the deep soft tissues of the body, such as muscle, fat, or connective tissue. It can occur in the extremities (arms and legs), trunk, or retroperitoneum (the space behind the abdominal cavity). Soft tissue angiosarcomas are often larger and more aggressive than cutaneous angiosarcomas.

• Primary soft tissue angiosarcoma: This arises spontaneously without any known predisposing factors.
• Secondary soft tissue angiosarcoma: This can occur after previous trauma, surgery, or exposure to certain chemicals.

Angiosarcoma can also occur in certain parts of the body including:

• Breast: This is a rare type of breast cancer that originates in the blood vessels of the breast.
  • Primary breast angiosarcoma: This arises spontaneously in the breast tissue. It's more common in younger women than typical breast cancer.
  • Secondary breast angiosarcoma: This can occur after radiation therapy for breast cancer (similar to radiation-associated angiosarcoma of the skin).
• Liver: This is a very rare and aggressive cancer that originates in the blood vessels of the liver. It's often associated with exposure to certain chemicals, such as vinyl chloride (used in the production of PVC plastics), arsenic, or Thorotrast (a radioactive contrast agent formerly used in medical imaging). However, in many cases, the cause is unknown.
• Angiosarcomas can rarely occur in other organs, such as the spleen, bone, heart, or gastrointestinal tract. These are extremely rare and often present unique diagnostic and treatment challenges.

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Angiosarcoma treatment

Given how uncommon and fast-growing angiosarcoma can be, tailored treatment that is focused on the cancer’s location, size, and stage, along with the patient's general health is crucial. AHN approaches treatment with a multidisciplinary approach involving specialists from a variety of medical backgrounds to develop a personalized treatment plan that is patient-focused.

Surgery

Surgery for angiosarcoma aims to remove the tumor completely with a margin of healthy tissue. This means that enough healthy tissue remains so the risk of the cancer returning is smaller. The specific surgical approach depends on the angiosarcoma's location and size. Surgical removal can be challenging depending on the location of the tumor and its proximity to vital structures. For example, angiosarcomas in the head and neck can be difficult to resect completely without causing significant cosmetic or functional problems. AHN focuses on the following surgical options:

• Primary treatment: Surgery is often the primary treatment for angiosarcoma, especially when the tumor is localized and can be completely removed.
• Wide local excision: The goal is to remove the tumor along with a wide margin of surrounding healthy tissue to ensure that all cancer cells are removed. This is crucial for preventing local recurrence.
• Lymph node dissection: If there is evidence that the cancer has spread to nearby lymph nodes, they may be removed surgically. A sentinel lymph node biopsy may be performed to determine if the cancer has spread to the first lymph node(s) in the drainage pathway.
• Amputation: In rare cases, amputation may be necessary for angiosarcomas of the extremities, particularly if the tumor is large, involves critical structures, or recurs after previous treatments.

Radiation therapy

Radiation therapy plays a significant role in the management of angiosarcoma. Its purpose is to use high-energy rays to damage and destroy cancer cells. The timing and application of radiation treatment are versatile and tailored to each patient's specific situation. Radiation may be used in several ways:

• External Beam Radiation Therapy (EBRT): The most common type of radiation therapy, in which radiation is delivered from a machine outside the body.
• Brachytherapy (Internal Radiation Therapy): Radioactive seeds or sources are placed directly into or near the tumor. This allows for a higher dose of radiation to be delivered to the tumor while sparing surrounding healthy tissues.
• Palliative radiation: When a cure isn't possible, radiation can be used to relieve symptoms like pain or bleeding, improving the patient's quality of life.

Chemotherapy

Chemotherapy is a vital part of the treatment strategy for angiosarcoma, a rare and aggressive cancer. It utilizes powerful drugs that travel through the bloodstream to target and destroy cancer cells throughout the body. In the context of angiosarcoma, chemotherapy can be employed at various stages of treatment:

• Doxorubicin (Adriamycin): A commonly used chemotherapy drug for angiosarcoma.
• Ifosfamide: Often used in combination with doxorubicin.
• Paclitaxel (Taxol): Another commonly used chemotherapy drug, particularly for cutaneous angiosarcoma.
• Docetaxel (Taxotere): Similar to paclitaxel.
• Gemcitabine: May be used in combination with other chemotherapy drugs.

Targeted therapy

Targeted therapy is a type of cancer treatment that uses drugs to specifically target cancer cells without harming normal cells. It works by identifying and attacking specific molecules or pathways that are essential for cancer growth and survival. With angiosarcoma, targeted therapy may be considered based on the specific characteristics of the tumor and the patient's overall health. Angiosarcoma tumors can be analyzed to identify specific genetic mutations or protein abnormalities that drive their growth. If a targetable abnormality is found, a targeted therapy drug that inhibits that specific target may be used to treat the cancer. Some potential targets for targeted therapy in angiosarcoma include:

• Vascular endothelial growth factor (VEGF) and VEGF receptors (VEGFRs): These proteins play a crucial role in angiogenesis, the formation of new blood vessels, which is essential for tumor growth and metastasis. Targeted therapies that inhibit VEGF or VEGFRs, such as bevacizumab and sorafenib, may be used to block angiogenesis and slow down cancer progression.
• Other tyrosine kinases: Tyrosine kinases are enzymes involved in cell signaling and growth. Some angiosarcomas may have mutations or overexpression of specific tyrosine kinases, making them potential targets for targeted therapy.

Immunotherapy

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Angiosarcoma FAQs

Facing an angiosarcoma diagnosis can bring about emotions and questions. You may feel overwhelmed. Your AHN care team of specialists is here to help and will be with you every step to answer questions and talk with you about your options. To help you feel informed and give a place to start, we’ve included some frequently asked questions that may help guide your conversations with your care team.

What is the survival rate for angiosarcoma?

The survival rate for angiosarcoma varies greatly depending on factors such as the location of the tumor, the stage at diagnosis, the patient's overall health, and the treatment received. Generally, angiosarcoma is an aggressive cancer. The five-year survival rate can range from 20% to 60% depending on the individual’s specific health outcomes. Your AHN care team is here to help you navigate your angiosarcoma diagnosis with specific answers based on your health.

How treatable is angiosarcoma?

Angiosarcoma can be challenging to treat due to its aggressive nature and tendency to spread. However, it is treatable, and various treatment options are available:

• Surgery: This is often the primary treatment for localized angiosarcoma. The goal is to remove the entire tumor with clear margins (no cancer cells at the edge of the removed tissue).
• Radiation therapy: This may be used after surgery to kill any remaining cancer cells in the area. It can also be used as the primary treatment when surgery is not possible.
• Chemotherapy: This is often used for advanced or metastatic angiosarcoma, and sometimes in combination with surgery and/or radiation.
• Targeted therapy: As mentioned earlier, targeted therapy may be used if the tumor has specific genetic mutations or protein abnormalities that can be targeted by drugs.
• Immunotherapy: This is an emerging treatment option that may be considered, especially in the context of clinical trials.

What does an angiosarcoma look like?

The appearance of angiosarcoma can vary depending on its location and stage:

• Skin: Angiosarcoma often appears as a bruise-like or reddish-purple patch or nodule on the skin. It may also look like a cluster of small, raised bumps. Over time, the lesion may grow, ulcerate, and bleed.
• Soft tissue: Angiosarcomas in deeper tissues may not be visible externally. They may present as a growing mass or cause pain and swelling in the affected area.
• Internal organs: Angiosarcomas in internal organs may be difficult to detect until they cause symptoms related to the organ's function.

What is the difference between hemangiosarcoma and angiosarcoma?

The terms "hemangiosarcoma" and "angiosarcoma" are often used interchangeably, but there can be a slight difference in usage:

• Angiosarcoma: This is the more general term for a malignant tumor arising from the endothelial cells that line blood vessels or lymphatic vessels.
• Hemangiosarcoma: This term is often used specifically when the angiosarcoma originates from blood vessels. In veterinary medicine, hemangiosarcoma is a common cancer in dogs, and that is where you will likely see the term used most often.

In practice, the terms are frequently used synonymously, and both refer to the same type of cancer.

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Contact us

To make an appointment you can:

• Call our scheduler at 412-359-8614
• Schedule online
• Use MyChart and schedule with Dr. Lisa Ercolano to make an appointment

Please bring all relevant imaging on CD or through PowerShare with correlating reports for review by our team to your initial appointment.

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