Accounting for only 1-2% of soft tissue sarcomas, angiosarcoma is not one of the common sarcomas. It is very simply put, a malignant tumour of connective tissue. The name “angiosarcoma” indicates what the cancer is. A “sarcoma”, essentially means a tumour originating from the connective tissue and “angio” means relating to blood vessels. Therefore, an angiosarcoma is a destructive malignancy of cells lining and forming the blood vessels. These cells are also known as the “ENDOTHELIAL CELLS”.
To start with, how is an angiosarcoma caused ?
Even though most cases are sporadic, meaning they occur randomly, without a known exposure or cause, there are also cases which have been linked to chronic exposure of certain environmental agents.
These important environmental predisposing conditions include ;
- Radiation- especially after exposure to radiation therapy for other cancers.
Radiation associated angiosarcoma typically is seen several years post therapy, especially seen after treatment for breast cancer.
- Exposure to chemical toxins (eg, vinyl chloride, arsenic)
It can also be seen in certain conditions of the body such as ;
- Chronic Lymphedema (it is not directly evident how blockage of lymph node drainage contributes to angiosarcoma)
- Pre-existing conditions of the body like arteriovenous fistulas.
Where does angiosarcoma occur ?
Angiosarcoma can occur just about anywhere in the body however they classically arise in the skin or subcutaneous soft tissues, just below the skin. They are seen as cutaneous tumors, meaning they are seen occurring in the skin of the scalp or face of elderly patients. However, angiosarcoma can have an extensive anatomic distribution including organs besides skin and cutaneous tissue such as deep soft tissue, breast, visceral organs and osseous locations like bone.
What are the clinical features of angiosarcoma ?
The clinical presentation of angiosarcoma totally depends on its area of occurrence. In the common type of angiosarcoma, the tumour starts as a bluish-purplish bruise or a red nodular rash. Cutaneous tumours frequently have small satellite lesions adjacent to the main tumour.
Regrettably, due to the harmless appearance of the early dermal lesion, it is easy to miss noticing the lesion and as a consequence develop systemic or widespread disease by the time of final diagnosis. If angiosarcoma is affecting the visceral organs of the body or the heart, it mostly causes that failure of that particular organ.
Clinically, angiosarcoma is very aggressive, and it is important to control the disease before it becomes disseminated. It is also known to be highly infiltrative, beyond its clinically apparent extent and often multifocal, therefore local control is associated with a high failure rate.
What are the grades for angiosarcoma ?
After removing an angiosarcoma, surgically, the tissue is submitted for histopathological examination, to establish diagnosis. Histopathologically, an angiosarcoma is seen occurring in a spectrum. It can range from the benign, well-differentiated tumours with semblance of blood vessels to malignant, poorly differentiated tumours with no resemblance to the blood vessels/ tissue of origin.
Low grade lesions resemble the endothelial cells they originate from hence they are well differentiated tumours. They show tumour blood vessels, which vary from normal blood vessels, in that they are atypical and proliferative.
High-grade lesions, on the other hand, do not resemble the endothelial cells from which they arise, hence can be confused with other diseases and need advanced diagnostic tests to be confirmed.
How can angiosarcoma be diagnosed ?
The initial screening tests are Ultrasonography, MRI and CT- scanning. Definitive diagnosis is made by biopsy and immunohistochemistry. Immunohistochemisrty is a diagnostic test wherein we test for antigens & antibodies of the disease. Positive immunohistochemical testing for the endothelial cell markers: CD31, CD34, and Factor VIII-antigen (which are all blood vessel markers) confirms angiosarcoma.
What can angiosarcoma be confused with ?
Cutaneous angiosarcoma can initially be misdiagnosed as bruising, infections of the skin, and sometimes even allergies or insect bites. What will differentiate these from angiosarcoma is the duration of the lesion and the way that it spreads.
Cutaneous angiosarcoma is also similar in appearance to another disease called Kaposi’s sarcoma. Both Kaposi sarcoma and angiosarcoma are derived from endothelial cells and are mostly multicentric in nature, involving skin but only Kaposi sarcoma is positive for its causative virus- the Human Herpes Virus 8.
What is the treatment for angiosarcoma ?
The first line of treatment for primary angiosarcoma is surgery. This is followed by high-dose radiotherapy either alone or combined with chemotherapy. Yet, the survival rates that are being obtained are still not satisfactory.
Surgery is the major foundation for the treatment of primary angiosarcoma. However, surgery can only control the disease locally and is not the treatment of choice for widespread disease. The surgical guidelines call for total surgical resection of the tumour with wide clear margins to reduce the recurrence of angiosarcoma.
Having clear margins basically means removing the tumour completely, along with some normal, unaffected tissue adjacent to the tumour. This will enforce that the tumour is completely removed. The problem with local surgery is that angiosarcoma is multicentric and its presence in vital organs can make complete tumour resection difficult.
Relevant investigations should be performed in due time, contributing to a accurate diagnosis and appropriate immediate operative treatment.
How to find and reach cancer specialists for the treatment of angiosarcoma ?
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Disclaimer: The content provided here is meant for general informational purposes only and hence SHOULD NOT be relied upon as a substitute for sound professional medical advice, care or evaluation by a qualified doctor/physician or other relevantly qualified healthcare provider.
