Overview
Angiosarcoma is a very rare and aggressive type of soft tissue cancer that originates from the cells that line the walls of blood vessels or lymph vessels. This type of cancer can appear in any part of the body; however, it most commonly manifests on the skin, particularly in the head and neck area.
Because it originates from vascular structures, angiosarcoma cells may tend to spread to other parts of the body through the bloodstream. While it is usually seen in older adults, it can affect any age group.
The course of the disease may vary from person to person depending on the location of the tumor, its size, and how early it is caught.
A professional oncological approach aims to control the spread of the tumor and preserve the patient's quality of life.
Symptoms of angiosarcoma
Symptoms of angiosarcoma may change depending on which part of the body the tumor develops in. Types located on the skin usually start with visible changes, while types in internal organs may lead to more general health complaints.
- Skin color changes: Purplish, dark blue, or reddish patches that may look like a bruise but do not heal can be seen in the affected area.
- Swelling and mass formation: Lumps that grow on or just under the skin surface and can sometimes be painful to the touch may be noticed; these masses may grow over time and press on surrounding tissues.
- Bleeding and ulceration: The skin in the area of the tumor may become sensitive and can bleed even with light impacts or become an open sore (ulcer).
- Pain due to internal organ involvement: Angiosarcomas developing in organs like the liver or heart can cause a feeling of discomfort, fullness, or a dull pain in that region.
- Edema and bloating: In cases where lymph vessels are affected, significant swelling may develop due to fluid accumulation, especially in the arms or legs.
When to see a doctor
If you notice a patch on your skin that does not heal, changes color, or grows rapidly, it may be recommended to consult a dermatologist or surgeon without losing time. Specifically, new skin changes developing in areas that have previously received radiation therapy should be examined carefully.
If you experience sudden severe shortness of breath, chest pain, or unbearable pain in the abdominal area, you should call emergency services immediately as this could indicate a complication created by the tumor in internal organs.
Causes of angiosarcoma
While the exact cause of angiosarcoma cannot be fully determined in most cases, it is known that mutations occurring in the DNA of vascular cells, leading to uncontrolled proliferation, start this process. Certain environmental and medical factors may contribute to this increased risk.
- Radiation therapy: Radiotherapy received for another type of cancer (e.g., breast cancer) may trigger the development of angiosarcoma years later by causing damage to the vascular cells in that area.
- Chronic lymphedema: Long-term accumulation of lymph fluid in an area and persistent swelling (e.g., arm swelling after mastectomy) can lay the groundwork for the vascular structures in that region to become cancerous.
- Exposure to chemicals: Types such as liver angiosarcoma can emerge as a result of contact with certain industrial chemicals like vinyl chloride, arsenic, or thorium dioxide.
- Genetic predisposition: In rare cases, certain hereditary syndromes (e.g., neurofibromatosis) can increase a person's risk of developing these types of vascular cancers.
Diagnosis of angiosarcoma
The diagnosis process is carried out by combining tissue samples taken from the suspicious area and advanced imaging methods. Determining the stage of the cancer is the most important step of the treatment plan.
- Tissue biopsy: A small piece taken from the suspicious mass or skin patch is examined in a pathology laboratory; this is the most definitive way to diagnose angiosarcoma.
- Imaging tests (MRI and CT): These are used to determine the size, depth, and relationship of the tumor with surrounding tissues; they can also check whether the cancer has spread to other organs.
- PET scan: By determining the activity of cancerous cells in the body, it can help detect areas where the disease has spread (metastasis).
Treatment of angiosarcoma
Angiosarcoma treatment is planned specifically for the individual according to the location and extent of the tumor. Usually, a combined approach using multiple treatment methods is preferred.
- Surgical intervention: The main treatment method is usually the surgical removal of the tumor and some healthy tissue around it; the goal is to leave no cancerous cells in the body.
- Radiotherapy: Using high-energy beams, the aim may be to destroy remaining cells after surgery or to shrink the tumor in cases where surgery is not possible.
- Chemotherapy: Drug therapy can be used to stop cells from proliferating, especially in cases where there is a risk of the cancer spreading to other parts of the body.
- Immunotherapy and targeted therapies: New generation treatments that stimulate the body's immune system against cancer cells or block specific mechanisms that allow the tumor to grow can be tried.
Risks of angiosarcoma
The aggressive nature of angiosarcoma can lead to certain risks and complications during and after the treatment process. Management of these risks requires regular follow-up.
- Metastasis (spread): Angiosarcoma cells can spread through the blood to the lungs, liver, or bones, potentially disrupting the functions of these organs.
- Local recurrence: There may be a possibility of the cancer reappearing in the area where the tumor was removed; therefore, check-ups at frequent intervals may be necessary.
- Organ function loss: Depending on the location of the tumor (e.g., if it is in the heart), risks of heart failure or serious internal bleeding may develop.
Prevention of angiosarcoma
Since most angiosarcoma cases develop due to random genetic mutations, it may not be possible to prevent it entirely. However, avoiding known risk factors can minimize the risk.
- Lymphedema management: Early treatment of swelling (lymphedema) developing after surgery or radiation and controlling it with massage/compression methods may reduce associated risks.
- Chemical protection: It can be of vital importance for people working with chemicals known to be carcinogenic in the workplace to use protective equipment.
- Early awareness: For people who have received radiation therapy, regular monitoring of skin changes in the treatment area and consulting a doctor in suspicious cases is the most effective prevention method.
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2- Primary cardiac angiosarcoma - a review https://pubmed.ncbi.nlm.nih.gov/24452054/
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4- An Update on Cutaneous Angiosarcoma Diagnosis and Management https://pubmed.ncbi.nlm.nih.gov/39042137/
5- Angiosarcoma of the breast: A review https://pubmed.ncbi.nlm.nih.gov/38318005/