Angiosarcomas
Angiosarcomas are a rare and aggressive form of cancer that originates from blood vessels, with the potential to develop in various parts of the body, most commonly in the skin and soft tissues of the arms, legs, neck, and head. Individuals with specific risk factors, such as previous exposure to radiation or chemotherapy, chronic lymphedema, and certain genetic disorders, are at a higher risk for developing these tumors. Often, angiosarcomas may arise as complications from existing conditions, including surgeries or the presence of foreign materials in the body.
These tumors can manifest as growing masses in soft tissues or as blue or black nodules on the skin. Diagnosis typically involves identifying abnormal growths, although some tumors in deeper tissues may remain undetected until they cause significant symptoms. Treatment strategies primarily include surgery and radiation, with chemotherapy using agents like doxorubicin also being an option. Despite treatment, angiosarcomas are known for their rapid spread and high recurrence rates, leading to a challenging prognosis, with five-year survival rates reported between 20 to 35 percent. Understanding the nature and risks associated with angiosarcomas is crucial for early identification and management.
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Subject Terms
Angiosarcomas
RELATED CONDITIONS: Soft-tissue malignancy
DEFINITION: Angiosarcomas are cancers that develop from blood vessels. Although the most common sites where they occur are the arms, legs, neck, and head, these tumors can occur anywhere on the body, most frequently on the skin or in the soft tissue. Angiosarcomas are made up of malignant cells that rapidly proliferate and are capable of extensive infiltration.
Risk factors: Some risk factors have been linked with the development of angiosarcoma. Primarily, these include previous exposure to radiation therapy or chemotherapy, as well as exposure to carcinogens such as certain polyester fibers or bone wax. Persons with chronic lymphedema are also at increased risk. Certain genetic disorders are also predispositions. These include bilateral retinoblastoma, Ollier disease, and Maffuci syndrome.
Etiology and the disease process: In many cases, angiosarcoma develops as a complication of a preexisting condition. For example, a radical mastectomy can lead to angiosarcoma in the soft tissue and skin of the breast. Additionally, foreign materials in the body, such as shrapnel, have resulted in angiosarcomas.
Incidence: Angiosarcoma is a very rare tumor type, occurring in only two to three patients per one million individuals. Angiosarcoma can afflict a person at any age. Those 60 years and older are more likely to develop the illness. Nearly half of all angiosarcomas develop in the head and neck regions, but they also can occur in the soft tissue of the lower and upper extremities. Less common are those that occur in other areas of soft tissue, such as the breast, kidney, and liver.
Symptoms: Angiosarcomas in the soft tissues of the extremities may be identified as growing masses, while those that occur on the skin generally appear as blue or black nodules. Angiosarcomas that develop in other areas of soft tissue, especially within the abdominal cavity, are capable of growing quite large and often are not diagnosed until the size of the tumor begins to affect the surrounding tissues and organs.
Screening and diagnosis: Diagnosis occurs by recognizing the abnormal growths within the skin or extremities of the patient. Angiosarcomas within the abdominal cavity may remain undiagnosed until related symptoms become apparent, such as neuronal symptoms occurring from compression of the lumbar or pelvic nerves.
Treatment and therapy: Surgery and radiation therapy are common methods of treating angiosarcomas. Depending on the location, chemotherapy may also be used in combination with these other strategies. The most common chemotherapeutic agent for angiosarcoma is doxorubicin hydrochloride, sold in the United States under the trade name Adriamycin. As of mid-2020, various types of immunotherapies have been tested in combination with other treatments. None, however, had yet been proven to be practice-changing.
Prognosis, prevention, and outcomes: Angiosarcomas are aggressive tumors that spread rapidly and frequently reoccur, even after treatment. In 2022, the reported five-year survival rate for these patients is approximately 20 to 35 percent.
Bibliography
"Angiosarcoma." The Cleveland Clinic, 2024, my.clevelandclinic.org/health/diseases/22778-angiosarcoma. Accessed 19 June 2024.
Brennan, Murray F., et al. Management of Soft Tissue Sarcoma. New York, Springer, 2013.
Carsi, Maria, et. al. "Angiosarcoma." MedScape, 18 Aug. 2022, emedicine.medscape.com/article/276512-overview#a2. Accessed 19 June 2024.
Fayette, J., et al. "Angiosarcomas, a Heterogeneous Group of Sarcomas with Specific Behavior Depending on Primary Site: A Retrospective Study of 161 Cases." Annals of Oncology, Dec 2007, pubmed.ncbi.nlm.nih.gov/17974557. Accessed 19 June 2024.
Fury, Matthew G., et al. "A 14-Year Retrospective Review of Angiosarcoma: Clinical Characteristics, Prognostic Factors, and Treatment Outcomes with Surgery and Chemotherapy." Cancer Journal, May-Jun 2005, pubmed.ncbi.nlm.nih.gov/16053668. Accessed 19 June 2024.
Goldblum, John R., et al. Enzinger & Weiss's Soft Tissue Tumors. 6th ed. Philadelphia, Saunders, 2014.
Singla, Smit, et al. "Challenges in the Treatment of Angiosarcoma: A Single Institution Experience." American Journal of Surgery, 16 Apr. 2014, doi.org/10.1016/j.amjsurg.2014.01.007, Accessed 19 June 2024.
"Soft Tissue Sarcoma-Symptoms and Causes." The Mayo Clinic, 27 Apr. 2023, www.mayoclinic.org/diseases-conditions/soft-tissue-sarcoma/symptoms-causes/syc-20377725. Accessed 19 June 2024.
Weiss, S. W., J. Lasota, and M. M. Miettinen. "Angiosarcoma of Soft Tissue." Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon, IARC, 2002.
Wood, Georgina, et. al. "Immunotherapy in Sarcoma: Current Data and Promising Strategies." ASCO Publications, 23 May 2024, ascopubs.org/doi/10.1200/EDBK‗432234. Accessed 19 June 2024.