What are the other Names for this Condition? (Also known as/Synonyms)

• Angiosarcoma of the Scalp
• Head and Neck Angiosarcoma
• Wilson Jones Angiosarcoma of the Face and Scalp

What is Angiosarcoma of Skin? (Definition/Background Information)

• Angiosarcoma of Skin is an infrequent, aggressive, and often rapidly developing tumor, without signs and symptoms in the early stages. The tumor arises from the vascular linings, which involve the blood vessels
• They are regarded as a type of high-grade malignant cancer that spreads and infiltrates the surrounding tissues and organs. “High-grade” means that the tumor is very aggressive
• The outcome for an individual with this tumor is poor, due to its high metastasizing rate. Metastasis is when the tumor leaves the site of origin and grows in another site
• Skin Angiosarcomas are seen to peak during late adulthood. These tumors are normally painless and located on the skin; common sites being the face and scalp. For this reason, this tumor is also known as Wilson Jones Angiosarcoma of the Face and Scalp
• A combination of chemotherapy (drugs), radiation therapy, and surgical procedures, are used to treat these skin tissue tumors

Who gets Angiosarcoma of Skin? (Age and Sex Distribution)

• Angiosarcoma of Skin affects a wide range of age groups, but it normally peaks after the age of 60 years
• Infants and young children are usually not affected
• Males are affected more than females
• There is no known ethnic or racial preference, and the malignancy is observed worldwide

What are the Risk Factors for Angiosarcoma of Skin? (Predisposing Factors)

The risk of Angiosarcoma of Skin is linked to the following factors:

• A previously performed radiotherapy for other cancer conditions - the tumor may be radiation-induced. In such cases, it is called Radiation-Induced Angiosarcoma. This is also referred to as “secondary”, because it is caused by the radiation that treated the original tumor. The radiation has an expected side effect of damaging the genetic material in the normal cells. This can lead to the development of a “secondary” cancer
• Chronic obstruction of the lymph vessels, called lymphedema, due to mastectomy (surgical removal of the breast or the lymph nodes under the armpit - a treatment for breast cancer). This can increase the risk for developing Skin Angiosarcoma
• The risk is greater in women who have undergone breast cancer surgery to remove large masses of breast tumors
• Chemical and environmental contaminants, such as arsenic, thorium dioxide, vinyl chloride, dioxin, and many other agents
• Presence of foreign materials in the body, such as graft material (plastic or steel), bone wax, polyester fiber (Dacron from sutures), etc., which are sometimes known to act as carcinogenic (cancer-causing) agents
• Angiosarcoma of Skin has also been associated to occur in patients diagnosed with neurofibromatosis (type 1), Klippel-Trenaunay syndrome, and Maffucci syndrome
• Individuals affected by immune deficiency disorders, such as AIDS, have a higher risk of developing the tumor

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Angiosarcoma of Skin? (Etiology)

• The exact cause and mechanism of formation of Angiosarcoma of Skin is presently unknown. They are thought to occur as a reactive process owing to some preexisting condition
• These preexisting conditions and certain situations include:
  • Radiation exposure
  • Chronic lymphedema
  • Exposure to chemicals and environmental pollutants
  • Presence of foreign materials in the body for long periods (such as an implant or graft)

What are the Signs and Symptoms of Angiosarcoma of Skin?

The signs and symptoms of Angiosarcoma of Skin include:

• The presentations are based on the location of Skin (or Cutaneous) Angiosarcomas - common sites being the face, scalp, and extremities. Other affected regions include the head and neck, arm, and torso
• The location of the skin tumor may be a site of previous radiation therapy, undertaken for some other cancer type
• The skin tissue malignant tumors initially grow at a moderate rate, and then suddenly start progressing rapidly during the later stages. In most cases, the initial tumor growth periods are asymptomatic
• The tumors may appear as skin bruises or skin ulcers; a lump-like nodule may be observed, which may be occasionally painful. A single nodule or multiple nodules can occur in the same area
• Due to large size of tumor growth, the adjoining organs, nerves, and muscles, may be compressed or restricted. Often, these signs may be the first indications of the tumor
• In some individuals bleeding disorders, anemia (decreased red blood cells), internal bleeding, and liver damage, may be observed - these developments may be sudden or spontaneous
• Enlarged lymph nodes

How is Angiosarcoma of Skin Diagnosed?

The diagnostic tests vary, based on location of the tumor. A diagnosis of Angiosarcoma of Skin may involve:

• Physical examination and evaluation of the patient’s medical history
• Histopathological studies conducted on a biopsy specimen - the specimen is examined under the microscope by a pathologist to arrive at a definitive diagnosis
• MRI and CT scans to aid in obtaining a clear image of the tumor prior to surgery
• PET scans may be used to determine the extent of tumor spread in the body, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Angiosarcoma of Skin?

The complications of Angiosarcoma of Skin may include:

• Usually by the time a Skin Angiosarcoma is detected, it is likely that it has grown and metastasized aggressively, damaging organs and tissues beyond repair. Frequent sites of metastasis are the lungs, lymph nodes, liver, and bones
• Cutaneous angiosarcomas are known to have a high recurrence rates following their surgical excision and removal
• Blood loss during invasive treatment methods may be heavy
• Damage of vital nerves, blood vessels, and surrounding structures may take place during surgery
• Side effects from chemotherapy (toxicity) and radiation therapy

How is Angiosarcoma of Skin Treated?

The treatment of Angiosarcoma of Skin may be undertaken as follows:

• Any combination of chemotherapy, radiation therapy, and invasive procedures (surgery) are used to treat Skin Angiosarcomas
• Embolization (clotting the vessels in the tumor) is used to provide temporary relief from the symptoms, and reduce blood loss during a surgical procedure
• Wide surgical excision of Skin Angiosarcoma with removal of the entire lesion is the standard treatment method considered
• When these angiosarcomas are at an inaccessible location, or it is unsafe for surgical intervention, non-invasive procedures, such as chemotherapy and radiation therapy, may be adopted
• Post-operative care is important until the surgical wound heals
• Follow-up care with regular screening and check-ups are important

How can Angiosarcoma of Skin be Prevented?

Currently, there are no definitive methods to prevent the formation of an Angiosarcoma of Skin. However, certain general precautions may be considered that can help decrease the risk for cancer.

• Adopt a lifestyle that avoids prolonged exposure to pollutants and carcinogens
• Healthy diet and physical activity/exercise

What is the Prognosis of Angiosarcoma of Skin? (Outcomes/Resolutions)

• Skin Angiosarcomas are rare and highly malignant cancers that have not yet been completely understood. The long-term prognosis depends on a combination of factors such as:
  • Age of the individual
  • Tumor stage at detection
  • Tumor size and location
  • Its ki-67 value - a protein found in cells that is a good indicator of how fast the tumor cells are growing. The ki-67 value is determined by a pathologist and is usually mentioned in the pathology report
  • Patient’s response to treatment and medical therapy
• Given the high rate of recurrence and metastasis, the long-term outcome (5-year) is poor for most individuals
• The best treatment practice for Angiosarcoma of Skin involves a combination of radiotherapy with surgical intervention

Additional and Relevant Useful Information for Angiosarcoma of Skin:

Angiosarcomas are classified into the following three major types:

• Angiosarcoma of skin
• Angiosarcoma of breast
• Angiosarcoma of spleen
• Angiosarcoma of liver
• Angiosarcoma of soft tissue