What Is Angiosarcoma

Overview

Angiosarcoma is a Latin name for a tumour of blood vessels. While "sarcoma" in Latin means the mass, "Angio" means the blood vessels. It affects the inner lining and sensitive layer of the blood vessels, endothelium, which is in direct contact with the blood. It constitutes about 2% of all the soft tissue tumors in the body, which form 1% of the tumors in the body. This urges us to know why, where, and how it is formed.

Regarding the site, angiosarcoma affects the narrow blood vessels where a contact of the blood flow may be more impressive on the wall of the blood vessels. Consequently, it can affect any blood vessels with less appearance in the large blood vessels and the heart. Angiosarcoma, for example, constitutes up to 5% of the skin soft tissue tumors with more predilection for the head and neck. Angiosarcomas of the liver, brain, breast and lungs have been reported.

Causes of angiosarcoma

Based on this previous generalized location, causes beyond angiosarcoma have been a matter of study and debate. While some studies related the appearance of angiosarcomas to breast cancer-linked genes such as BRCA 1&2, others attributed it to exposure to radiation, even for the treatment of other cancers. On one hand, Some studies referred to it as a result of lymphatic vessel obstruction, caused by parasites such as filariasis. On the other hand, some other studies referred their causes to the exposure to toxic heavy metals such as arsenic, radium and even body-building steroids.1

Signs and symptoms of angiosarcoma

The diagnosis of angiosarcoma should involve a history of one of its possible causes in addition to a possible previous history of lymph node removal. Also, lymphatic obstruction with a rare positive family history has been implicated.2 The patient begins to experience the following:

  • Red elevated  localized mass of the skin
  • Skin ulcer or fungal mass that easily bleeds
  • Its occurrence in internal organs may manifest by obstruction of nearby vessels or compression effect by the mass weight
  • Affecting other organs by blood spread is a possibility since it normally has more than one centre all over the body

Management and treatment for angiosarcoma

The choice of managing angiosarcoma falls among several stages and follows several considerations due to its high aggressiveness and rapid progression.3 This would include,

  • Surgical intervention is the first choice - removal of the tumour with a safety margin. However, the high rate of recurrence and the difficulty of localizing the tumour in old people may be a factor to avoid considering surgery as the first choice
  • Radiotherapy is one of the options, especially with poorly identified margins as in the elderly. Yet, it can be used after the surgery to ensure the complete treatment of the tumour
  • Chemotherapy, although not the most affecting, proved to decrease the rate of recurrence on the usage of two or more therapeutic compounds
  • Immune-based therapy can be used as programmed cell death. However, its usage in treating angiosarcoma is still not as widely based as its usage in treating other malignant tumours
  • Target therapy can be used which affects the origin of the tumour, in this case, it is the blood vessels. This may involve inhibition of the growth of the malignant blood vessels and enhancing the replacement of the malignant blood vessels by new ones, using some vascular growth factors

FAQs

How is angiosarcoma diagnosed

The diagnosis involves the clinical picture as well as some imaging techniques for the blood vessels or the tumour masses. For instance, ultrasonography, computerized tomography scan, and magnetic resonance imaging can be used to determine the blood vessel wall and its delineation.

How can I prevent angiosarcoma

In talking about angiosarcoma, we have to consider its risk factors which include:

  1. Exposure to heavy metals such as arsenate, as for chemists in a laboratory
  2. Radiation exposure, especially those working with radioactive materials or living near radio emissions
  3. People with angioedema
  4. People with tropical parasitic diseases such as filariasis
  5. Some familial diseases
  6. People with a previous history of cancer and radiotherapy

Who are at risk of angiosarcoma

The previous risk factors show the ability to modify some of them regarding their causality. For example: 

  • Applying safety precautions for radiation exposure as well as heavy metals
  • Monitoring the people with familial disease
  • Applying supportive measures to avoid angioedema would be a super plus
  • Treating varicosities surgically or medically

How common is angiosarcoma

Angiosarcoma represents 1 to 2% of all sarcomas. It affects the risk factor groups as well as mostly the people over the age of 70 years.

When should I see a doctor

While the medical healthcare systems are still recovering from the pandemic effects, medical consultation is needed to be in limited conditions. The presence of one of the previous risk factors in addition to suspicious forms of skin affection as described in the clinical picture would be the most eligible for medical examination. Not only this, the sudden and persistent appearance of bleeding lesions on the skin would urge the demand for medical consultation.

Summary

Angiosarcoma is a rapidly aggressive malignancy that needs a high awareness of its risk factor for early intervention rather than prevention. This would contribute to lower mortality rates and decreased risk ratio.

References

  1. Spiker AM, Mangla A, Ramsey ML. Angiosarcoma. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 [cited 2023 Jan 30]. Available from: http://www.ncbi.nlm.nih.gov/books/NBK441983/
  2. Pawlik TM, Paulino AF, McGinn CJ, Baker LH, Cohen DS, Morris JS, et al. Cutaneous angiosarcoma of the scalp: a multidisciplinary approach. Cancer. 2003 Oct 15;98(8):1716–26.
  3. Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ. Angiosarcoma. Lancet Oncol. 2010 Oct;11(10):983–91. 
This content is purely informational and isn’t medical guidance. It shouldn’t replace professional medical counsel. Always consult your physician regarding treatment risks and benefits. See our editorial standards for more details.

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Mohamed Abulfadl

Master of Medical Biochemistry and Molecular Biology- Faculty of Medicine, Aswan University, Egypt


Mohamed is a medical doctor with neurology and nephrology research interest. He has an experience
of working for three years as a dual specialist of diagnostic Medicine (both diagnostic imaging and
Laboratory medicine).
Additionally, he has an interest in supporting university students, either as a teaching assistant, mentor
or even invigilator since 2016.
He is currently on a PHD study on translational neuroscience in Bristol medical school in UK.

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