Under the term angiosarcoma, physicians summarize various malignant tumors of the vascular system of the skin. Angiosarcoma usually occurs only in people of advanced age (about 65 to 75 years), often after cancer treatment has already been given. The prognosis for angiosarcoma is rather unfavorable.
What is angiosarcoma?
Angiosarcoma occurs very rarely. About 1-2% of all new cancer cases are soft tissue tumors, and angiosarcoma new cases are correspondingly even rarer. Affected are almost exclusively people of older age, women are slightly more often affected than men. An angiosarcoma usually appears after a cancer treatment with radiation therapy, mostly after treatment of a breast carcinoma with a subsequent total surgery. In about 30% of all cases, angiosarcoma first appears in the area of the neck and head. Red spots appear, which after a short time turn bluish and in the further course “disintegrate” into minute tumors. Especially at the beginning, the spots are often misinterpreted and falsely diagnosed as hives or bruises, for example.
Causes
According to current research, angiosarcoma occurs more frequently after tumor treatment followed by radiation therapy. In particular, there is a clear association between the occurrence of breast carcinoma (breast cancer) with subsequent (ionizing) radiation therapy. People suffering from persistent lymphedema are also more likely to develop angiosarcoma. The cause may also be poisoning with some toxic substances, such as arsenic, vinyl chloride and thorotrast (contrast medium). An immunocompromised body due to certain viruses also favors the development of angiosarcoma. Kaposi’s sarcoma, which belongs to the family of angiosarcomas and mostly affects people suffering from HIV infection or AIDS, is quite well known. Quite significantly, skin that has been exposed to solar radiation several times is much more prone to angiosarcoma than skin that has not been exposed to any particular UV radiation.
Symptoms, complaints, and signs
Angiosarcoma can manifest itself through a variety of symptoms. Typically, there is redness of the skin and skin lesions. Most patients suffer from bruising and ulcers, which often occur all over the body and can cause accompanying symptoms such as pain and itching. Furthermore, angiosarcoma can lead to swelling of the upper abdomen, associated with a feeling of pressure in the affected region. Whether other signs occur depends on the size and location of the tumor. Smaller sarcomas can often be recognized only by small skin elevations and nodules under the skin that hurt when pressure is applied. Larger tumors can damage the tissues of the skin, causing injury and bleeding. In less severe cases, angiosarcomas cause hardening of the surrounding tissue. The skin then feels leathery and is less sensitive to pain. The tumor itself hurts to the touch, especially if it is located in the intimate area, around the face, or in other sensitive areas. Occasionally, pain or gastrointestinal discomfort is also present. If angiosarcoma is not treated, general symptoms such as fever and nausea may develop. As the disease progresses, the physical and mental well-being of those affected decreases significantly.
Diagnosis and progression
If the suspicion of angiosarcoma is confirmed, the physician will first try to find out whether the patient has been exposed to the typical sources of danger by taking a medical history. He or she will then perform a biopsy, which is the removal of tissue from the affected tumors. A laboratory physician will confirm the suspicion of angiosarcoma after subsequent cytological examination of the sample. The course of the cancer is rather unfavorable. Since angiosarcoma is initially unnoticed or in many cases not properly diagnosed at first, but at the same time spreads very rapidly through the blood vessels of the skin and metastasizes to many vital organs, it is difficult to treat. The 5-year prognosis is about 10% (the 5-year prognosis indicates how many of the patients with a given disease are still alive (tumor-free) after 5 years).
Complications
The outlook for disease with angiosarcoma is generally considered poor.Only up to a maximum of 24 percent of patients survive the first five years after surgery. The thickness of the tumor plays a far-reaching role. If the sarcoma is smaller than five centimeters, the prognosis may be somewhat more positive than in the case of large areal tumors, which originate in different parts of an organ and spread from there. In addition, age is a decisive factor. The older the patient, the lower his chances of survival. The prognosis is even worse for angiosarcoma that spreads from the vessels to the areas along chronic lymphedema, a lymphedema-associated angiosarcoma or Stewart-Trèves syndrome. Causes of death here are metastases to the lungs, pleura, and chest. However, the prognosis is poorest for angiosarcoma in the breast and for post-radiotherapy angiosarcoma. In the case of tumor in the breast, the chances of survival are only about ten percent. In most cases, life expectancy is slightly less than two years. In the case of tumor in the soft tissues, although 50 percent of those with the disease regrettably do not survive the initial period, fortunately of the other half nearly 34 percent reach the fourth year after surgery.
When should you see a doctor?
In the case of angiosarcoma, the affected person usually needs to see a doctor immediately. Because this is a cancer, early diagnosis and treatment are very important to prevent secondary damage and prevent the affected person from dying. In most cases, angiosarcoma is characterized by severe redness of the skin and the formation of bruises. Therefore, if these symptoms occur, a doctor must be consulted in any case. Also in case of swelling in the upper abdomen, a doctor should be consulted immediately, as this could be the cancer. As a rule, a dermatologist or a general practitioner can be consulted directly. In this case, further treatment must usually take place in a hospital. The dermatologist himself can remove the affected skin area. Furthermore, chemotherapy may be necessary for the affected person to prevent the cancer from spreading to other regions of the body. Life expectancy is reduced by angiosarcoma.
Treatment and therapy
House sites that show clear signs of angiosarcoma are radically removed by a dermatologist. However, excision can only be performed if the corresponding skin sites are not too large, otherwise the skin may not close optimally. The excision is followed by radiation therapy in combination with chemotherapy. Radiation and chemotherapy are intended to prevent any remaining tumor cells from growing and spreading again. Following this, immunotherapy may be sought. Despite available therapies, the prognosis is unfavorable because the rapidly spreading angiosarcomas have not infrequently already affected organs such as the liver and spleen. In addition, even after radical excision, new sarcomas form quite rapidly (recurrence), which in turn respond less well to drugs.
Outlook and prognosis
The prospects for recovery are very low in angiosarcoma. The onset of symptoms occurs mostly in people of advanced age, over 65 years. Due to age, the organism is already weakened at this point. In most cases, various previous diseases have existed for years and the body does not have the necessary resources for the necessary healing process. In addition, angiosarcoma is a sequelae of cancer. This means that in most cases the body is additionally weakened due to an operation as well as a subsequent cancer therapy. The treatment, which lasts for months, leads to a reduction in muscle strength as well as immune defences and is associated with numerous side effects. On its own, there is a lack of antibodies to achieve a cure of angiosarcoma. In addition to the low existing physical self-healing powers, a tiredness of life, despondency as well as a lack of mental strength are often to be expected. This is additionally not very conducive to the healing process. A positive attitude towards life, optimism and goals in life are elementary points in a recovery. About 1/3 of all patients who have undergone radiation therapy suffer from angiosarcoma.With current medical options for treatment, nearly all cases result in a fatal course of the disease with no chance of a cure.
Prevention
Angiosarcoma cannot be prevented. Patients after successful treatment of angiosarcoma must be followed closely thereafter. It is recommended that a checkup be performed approximately every three months. Patients with a relevant previous illness or patients who have frequently been exposed to toxic substances or who have frequently been sunburned should critically examine their skin themselves or visit a dermatologist regularly. At the first sign of angiosarcoma, the patient should be sure to report the suspicion to the attending physician. Otherwise, the general rules that everyone can follow apply in order to prevent cancer as far as possible. These include moderate use of nicotine and alcohol and a healthy diet.
Aftercare
If angiosarcoma is successfully treated, close follow-up care follows. Doctors recommend follow-up examinations every three months. Diagnosis is made by clinical and histologic findings. A single cure does not lead to immunity. As with other tumor diseases, patients can organize their daily lives in such a way that a recurrence is not encouraged. This includes avoiding nicotine, alcohol and other intoxicating substances. A healthy and balanced diet should be a matter of course. Low levels of physical exertion should be integrated into everyday life. In addition to the medical checks, the patient therefore has a high degree of personal responsibility. This also extends to regular critical examination of the skin surface. A doctor should be consulted immediately at the first signs of a recurrence of the disease. Realistically, it should be noted that follow-up care has little chance of success. After five years, only ten to twelve percent of sufferers are still alive. After a diagnosis, those affected should therefore deal with existential questions. It is not uncommon for new sarcomas to develop and spread to other parts of the body. Relatives should be prepared to care for a nursing case. Home arrangements should be adjusted. A nursing service should be hired.
What you can do yourself
When the typical skin changes or other symptoms of angiosarcoma are noticed, a physician must be involved. The patient can do several things to help medical treatment and minimize the risk for complications. First, strict personal hygiene applies. The area around the diseased skin is very sensitive and quickly becomes inflamed. Therefore, special care products from the drugstore should be applied in consultation with the doctor. Alternatively, preparations from nature can be used, for example calendula ointment or lotions with chamomile or lemon balm. Radiation therapy can be accompanied by a change in diet. In most cases, the treatment leads to a loss of appetite, which is why appetite-stimulating foods and drinks are recommended. However, stimulants such as alcohol or caffeine should be avoided. In addition, rest and bed rest are recommended. To ensure that no recurrences develop, the patient must visit the doctor regularly in the first weeks and months after treatment and have the skin sites examined. A complaints diary makes it easier for the physician to adjust the medication. If serious side effects and interactions occur, the medication must be discontinued immediately.
