Angiosarcoma
The content of the article:
- Causes and risk factors
- Forms of the disease
- Symptoms
- Diagnostics
- Treatment
- Possible complications and consequences
- Forecast
- Prevention
Angiosarcoma (hemangioendothelioma) is a rare malignant tumor originating from the endothelium (cells of the inner surface) of blood or lymph vessels. Differs in resistance to therapy and rapid metastasis.
Angiosarcoma can be localized in any part of the body and in any organ, but most often it affects soft tissues and skin (in 60% of cases). Other favorite sites of tumor localization are the mammary glands, spleen, bones and liver.
Angiosarcoma is a malignant tumor characterized by rapid metastasis
Angiosarcoma of the skin is diagnosed twice as often in men than in women; usually occurs in old age. Angiosarcoma of the liver is usually observed in men over 60 years old, and angiosarcoma of the mammary glands - exclusively in women 35-45 years old.
Causes and risk factors
The main cause of angiosarcoma is considered to be stagnation of lymph (lymphostasis). Violation of the outflow of lymph leads to the accumulation of carcinogenic substances in the tissues. By acting on endothelial cells, they become the cause of mutations, as a result of which malignant cells appear.
The risk of developing angiosarcoma increases when the body is exposed to thorium, radium, arsenic and its compounds, polyvinyl chloride.
The provoking factors also include:
- ionizing radiation;
- local immunity disorders;
- vascular factors.
The risk of liver angiosarcoma increases with the following diseases:
- Recklinghausen's disease;
- idiopathic chronic hemochromatosis.
The medical literature describes cases of the occurrence of cutaneous forms of angiosarcoma after mechanical trauma and viral skin lesions, against the background of benign angiogenic tumors.
Post-radiation angiosarcomas are a rare species. They sometimes develop 15-25 years after radiation therapy for malignant tumors (cancer of the cervix, breast, ovary, endometrium, penis, testicle).
Forms of the disease
There are several clinical forms of the disease:
- angiosarcoma of the extremities associated with lymphostasis (Stewart's syndrome);
- primary angiosarcoma of the mammary glands;
- idiopathic primary angiosarcoma of the scalp or face;
- post-radiation cutaneous angiosarcoma.
The rest of the angiosarcomas are classified according to their location (angiosarcomas of the heart, liver, kidney, etc.).
Symptoms
Malignant tumors caused by lymphostasis are usually localized on the lower limbs, the anterior chest wall, on the forearm, shoulder and in the ulnar fossa. They can be single or multiple. Initially, the tumor looks like a blue-violet spot, similar to a common hematoma. Then, in its place, a knot is formed, resembling a conglomerate of polyps, the surface of which is covered with erosions and ulcers and bleeds easily. The pathological process quickly spreads to the surrounding tissues.
Idiopathic cutaneous angiosarcoma is usually localized to the skin of the face, scalp, tonsils, pharynx or larynx, and the lower or upper jaw. At first, the swelling looks like an orange peel (looks like wrinkled, bumpy skin) or a hematoma. After this, painful nodes are formed, covered with erosion and prone to bleeding. This form of angiosarcoma rapidly metastasizes to the lungs.
Angiosarcoma of the mammary gland looks like a node with an uneven coloration from light blue to blood red.
Post-radiation angiosarcoma is usually located in the projection of the pelvis, chest or abdomen. In terms of clinical manifestations, it does not differ from other types.
In addition to local, there are general symptoms accompanying the development of angiosarcoma:
- weakness, fatigue;
- signs of intoxication (pallor, nausea, decreased overall health);
- increased body temperature;
- lack of appetite, weight loss;
- anemia.
Angiosarcoma of the liver is also characterized by:
- enlarged liver;
- abdominal pain;
- jaundice;
- ascites.
Angiosarcoma of the liver
In about every fourth case, patients with hepatic angiosarcoma develop massive bleeding into the abdominal cavity, quickly leading to death.
Diagnostics
For the diagnosis of angiosarcoma, a comprehensive laboratory and instrumental examination is carried out, including:
- general blood analysis;
- blood chemistry;
- a blood test for tumor markers;
- radiography;
- Ultrasound;
- computed or magnetic resonance imaging.
The final diagnosis is based on the results of the histological analysis of the tumor site obtained during the biopsy.
Treatment
The high degree of malignancy of angiosarcoma and its ability to rapidly metastasize to other organs make the therapy ineffective.
With early diagnosis of a malignant tumor, it is removed, after which radiation therapy is prescribed. In some cases, radiation therapy can also be used in the preoperative period. This combined approach reduces the growth of metastases, reduces the likelihood of relapses, and helps to alleviate the general condition of the patient.
In the presence of metastases in distant organs, chemotherapy is indicated.
Possible complications and consequences
Angiosarcomas quickly metastasize to the lungs, brain, bones, liver, kidneys, bones.
Forecast
The forecast is cautious. After the combined treatment of early stages of the disease, the five-year survival rate is within 45%, the ten-year survival rate is 25%.
Prevention
There are no specific measures for the prevention of angiosarcoma. Factors that contribute to an increase in general immunity play a certain role in preventing the disease:
- good nutrition;
- observance of the optimal regime of work and rest;
- rejection of bad habits;
- moderate exercise;
- general improvement of the body.
Elena Minkina Doctor anesthesiologist-resuscitator About the author
Education: graduated from the Tashkent State Medical Institute, specializing in general medicine in 1991. Repeatedly passed refresher courses.
Work experience: anesthesiologist-resuscitator of the city maternity complex, resuscitator of the hemodialysis department.
The information is generalized and provided for informational purposes only. At the first sign of illness, see your doctor. Self-medication is hazardous to health!
