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Angiosarcoma of Soft Tissue

Last updated April 30, 2018

Approved by: Krish Tangella MD, MBA, FCAP

CT findings of angiosarcoma of the diaphragm (tumors were located under the chest, a huge volume, heterogeneous enhancement, No mediastinal lymph nodes).

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

  • Hemangioblastoma
  • Lymphangioendothelial Sarcoma
  • Malignant Hemangiopericytoma

What is Angiosarcoma of Soft Tissue? (Definition/Background Information)

  • Angiosarcoma of Soft Tissue is an infrequent, aggressive, but 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
  • Soft Tissue Angiosarcomas are seen to peak during late adulthood. These tumors are normally painless and located deep inside the body tissues; common sites being the abdominal cavity, leg, arm, and trunk
  • A combination of chemotherapy (drugs), radiation therapy, and surgical procedures, are used to treat these soft tissue tumors

Who gets Angiosarcoma of Soft Tissue? (Age and Sex Distribution)

  • Soft Tissue Angiosarcoma 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

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

The risk of Angiosarcoma of Soft Tissue 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 arm pit; a treatment for breast cancer). This can increase the risk of developing Soft Tissue Angiosarcoma
  • Chemical and environmental contaminants, like arsenic, thorium dioxide, vinyl chloride,  dioxin, and many other agents
  • Presence of foreign materials in the body, like graft material (plastic, steel), bone wax, polyester fiber (Dacron, from sutures), etc., which are sometimes known to act as carcinogenic (cancer-causing) agents
  • Angiosarcoma of Soft Tissue 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, like 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 Soft Tissue? (Etiology)

  • The exact cause and mechanism of formation of Angiosarcoma of Soft Tissue, is unknown. They are thought to occur as a reactive process, owing to some preexisting factor(s)
  • These preexisting factors may 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 Soft Tissue?

The signs and symptoms of Angiosarcoma of Soft Tissue include:

  • The presentations are based on the location of Soft Tissue Angiosarcomas - abdominal cavity and lower limbs are common locations for this tumor
  • Other affected regions include, the head and neck, arm, and torso
  • The soft 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
  • 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, liver damage, etc. may be observed - these developments may be sudden or spontaneous
  • Enlarged lymph nodes

How is Angiosarcoma of Soft Tissue Diagnosed?

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

  • Physical examination, evaluation of patient’s medical history
  • Histopathological studies conducted on a biopsy specimen - the specimen is examined under a microscope by a pathologist to arrive at a definitive diagnosis
  • MRI scan, CT scan, 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

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 Soft Tissue?

The complications of Angiosarcoma of Soft Tissue may include:

  • Usually by the time a Soft Tissue Angiosarcoma is detected, it is likely that it has grown and metastasized aggressively, damaging organs and tissues beyond repair
  • This kind of angiosarcoma is also known to have a high recurrence rate, even after surgical excision and removal
  • Blood loss during invasive treatment methods may be heavy
  • Damage of vital nerves, blood vessels, and surrounding structures during surgery
  • Side effects from chemotherapy (toxicity), radiation therapy

How is Angiosarcoma of Soft Tissue Treated?

The treatment of Angiosarcoma of Soft Tissue is undertaken as:

  • Any combination of chemotherapy, radiation therapy, and invasive procedures (surgery), are used to treat Soft Tissue 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 the soft tissue tumor with removal of the entire lesion is the standard treatment mode
  • When Soft Tissue Angiosarcoma is at an inaccessible location, or it is unsafe for a surgical intervention; non-invasive procedures, such as chemotherapy and radiation therapy are adopted
  • Post-operative care is important: Minimal activity is allowed, until the surgical wound heals
  • Follow-up care with regular screening and check-ups are important

How can Angiosarcoma of Soft Tissue be Prevented?

  • There is no definitive method to prevent Soft Tissue Angiosarcoma occurrence; consider general precautions that decrease the incidence of cancer in the body
  • Adopting a lifestyle that avoids prolonged exposure to pollutants and carcinogens, either at home or at work, may help in preventing Angiosarcoma of Soft Tissue
  • Due to its high metastasizing potential and high recurrence rate, regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory for those who have already been treated for this tumor

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

  • Soft Tissue Angiosarcomas are rare and these highly malignant cancers 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 Soft Tissue involves a combination of radiotherapy with surgical intervention

Additional and Relevant Useful Information for Angiosarcoma of Soft Tissue:

Angiosarcomas can occur anywhere in the body; the most common tumors include the following:

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

What are some Useful Resources for Additional Information?

References and Information Sources used for the Article:

Helpful Peer-Reviewed Medical Articles:

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: March 8, 2014
Last updated: April 30, 2018