Angiosarcoma
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Angiosarcoma is a rare malignant vascular tumor. It grows aggressively and starts in blood vessels or lymphatic vessels. Together with Kaposi sarcoma, it belongs to the group of malignant vascular sarcomas. However, angiosarcoma differs in its origin, course, and growth pattern.
Angiosarcomas can occur in different forms. These include cutaneous angiosarcoma of the skin, post-radiation angiosarcoma after earlier radiotherapy, and angiosarcoma linked to chronic lymphedema. They mainly affect adults and can develop in almost any part of the body. They are often found in the skin, especially on the face and scalp, in soft tissue, or in organs such as the liver.
Doctors can also classify angiosarcomas by their tissue features. They mainly distinguish epithelioid, spindle cell, and differently differentiated forms. Epithelioid angiosarcoma contains round, epithelial-like tumor cells. It often has a more aggressive course. Spindle cell angiosarcomas show more elongated cell shapes.
Because angiosarcomas grow aggressively and invade nearby tissue, early diagnosis is very important.
Prof. Dr. med. Dipl.-Med. Holger Wehner
The treatment of angiosarcoma requires early diagnosis and an individually tailored therapeutic strategy. Complementary approaches such as hyperthermia may help support the body and improve quality of life throughout treatment.
Angiosarcoma Causes and Risk Factors
In many cases, doctors cannot find one clear cause of angiosarcoma. Angiosarcoma is a malignant vascular tumor that can arise from blood vessels or lymphatic vessels. Genetic changes in the vascular cells cause the cells to multiply without control. They can also grow into nearby tissue.
In general, angiosarcoma can be divided into primary and secondary forms. Primary angiosarcomas develop without a clear trigger. Secondary forms develop in connection with certain risk situations or previous tissue damage.
Important known risk factors and settings include:
- Chronic lymphedema
- Previous radiotherapy
- Certain chemical substances, such as vinyl chloride, arsenic, and formerly Thorotrast
- Ultraviolet radiation, especially in cutaneous angiosarcoma of the head and neck area
- Certain genetic or familial syndromes
- Older age, depending on subtype and location
It is important to understand that many people have no clear trigger. The key is to recognize known risk situations. Unusual changes should be checked early. This is important because angiosarcomas often behave aggressively.
Angiosarcoma Symptoms
Angiosarcoma symptoms can be very unclear at first. They mainly depend on where the tumor develops. In the early stages, cutaneous angiosarcoma may look harmless. It can resemble a bruise, a small swelling, or a skin change that does not heal well.
Over time, the lesion often becomes larger and more nodular. It may ulcerate or bleed. Deeper or internal angiosarcomas can cause local pressure symptoms, pain, or problems with the affected organ.
Possible symptoms of angiosarcoma include:
- Red-purple or bluish skin changes that first look like a bruise
- Swelling
- A lump that can be felt
- Local pain or a feeling of pressure
- Nodule formation
- Ulceration, oozing, or bleeding at the affected site
- General symptoms such as reduced performance, weight loss, or exhaustion
Angiosarcomas can also spread early. The tumor cells mainly spread through the bloodstream. The lungs are the most common site of metastases. The liver, bones, lymph nodes, and other organs may also be affected, depending on where the tumor started. Lung metastases often cause shortness of breath, chest pain, cough, or coughing up blood.
Typical warning signs include skin changes that look like a bruise but do not heal. They may grow, bleed, or become harder. New unexplained swelling, fast-growing lumps, or pain that lasts for a longer time should also be checked by a doctor.
Angiosarcoma Diagnosis
Diagnosing angiosarcoma can be difficult. Early symptoms are often very unspecific. Diagnosis usually starts with a careful clinical examination and detailed imaging. Imaging helps doctors assess the exact size, depth, and possible organ involvement.
Depending on the location, doctors may use ultrasound diagnostics, CT, or MRI. Imaging is especially important for deeper or internal angiosarcomas. It helps show the tumor and its relationship to nearby structures.
The final diagnosis is made through a tissue sample. A biopsy is usually taken and then examined under a microscope. Doctors assess whether the tissue is a malignant vascular tumor. They also use immunohistochemical markers. These markers help show endothelial features of the tumor cells. They also help distinguish angiosarcoma from other sarcomas or skin tumors.
After the diagnosis is confirmed by tissue testing, staging follows. This checks how far the disease has progressed. Because angiosarcomas can spread early, doctors often perform a CT scan of the chest and abdomen. The goal is to detect metastases early and adjust treatment to the individual situation.
Angiosarcoma Treatment and Prognosis
Angiosarcoma treatment aims to control the tumor, prevent the disease from progressing, and, depending on the stage, work toward cure. A multimodal approach is usually needed. This is because angiosarcomas are often very aggressive, grow into nearby tissue, and can spread early.
If the tumor is local, limited, and operable, treatment usually aims for complete surgical removal. This is often followed by adjuvant radiotherapy. Radiotherapy helps reduce the risk of recurrence. In some situations, systemic therapy may also be considered. This may be the case if the tumor is already very extensive.
If the tumor cannot be operated on, or if metastases are already present, treatment usually follows a palliative approach. The aim is to slow tumor growth, relieve symptoms, and improve quality of life. Depending on the subtype, different chemotherapy drugs, targeted therapies, or immunotherapies may be used. Palliative radiotherapy may also help. It can reduce pain, control bleeding, or shrink the tumor locally.
In addition to conventional medical treatment, complementary medicine is increasingly included in angiosarcoma care. This may include acupuncture to relieve pain or nausea during chemotherapy. It may also include psycho-oncology, adapted exercise therapy, and targeted nutritional support.
Hyperthermia is also being studied as an additional approach in angiosarcoma. However, the scientific evidence is still limited. In specific situations, especially together with repeat radiotherapy for radiation-associated angiosarcoma of the breast or chest wall, studies suggest better local tumor control. We use this potential and include hyperthermia as a synergistic part of our treatment concept. The aim is to improve blood flow in the tissue and make tumor cells more sensitive to conventional therapies. At the same time, we aim to support the well-being and quality of life of our patients as much as possible.
The prognosis of angiosarcoma mainly depends on the stage at diagnosis. It also depends on tumor size, whether complete removal is possible, and whether metastases are already present. Overall, angiosarcoma has an unfavorable prognosis. This is mainly because recurrence and distant metastases are common.
A cure is most likely when the tumor is found early, removed completely, and treated consistently with adjuvant radiotherapy. If angiosarcoma is already metastatic or cannot be operated on, long-term cure is much less common.
Dr. med. Karsten Ostermann M.A.
In angiosarcoma care, tumor stage, extent of disease, and individual patient factors should be carefully considered. An integrative approach can help support treatment comprehensively and alleviate burdensome symptoms in a targeted manner.
Further information
The information listed contains relevant topics and serves to improve understanding.