Kaposi's sarcoma is a variety of malignant neoplasms that appear on the skin. For the first time this pathology was described by the Hungarian dermatologist Moritz Kaposi, whose name today bears the name of this disease. Due to the specific appearance of the neoplasms, this pathology is sometimes called hemorrhagic sarcoma.
It must be said that the location of tumors is not limited to the skin, they often affect the internal organs along with the mucous membranes and lymph nodes. Next, we will find out what are the main causes of Kaposi's sarcoma, and also find out what symptoms are observed in patients and how treatment is carried out.
Causes of disease development
Kaposi's sarcoma is not very common among the population, but among HIV-infected people, especially men, it manifests itself in half of the cases. For certain, the reasons that cause the appearance of such neoplasms are not known today. But scientists confidently assume that this disease can developon the basis of the herpes virus of the eighth type, which is still insufficiently studied. Quite often, pathology can accompany other malignant processes in the form of mycosis fungoides, lymphogranulomatosis, lymphosarcoma, multiple myeloma and leukemia.
In order for this pathology to occur, a significant decrease in immunity is necessary due to various reasons. In addition, some categories of people have a much higher risk of developing Kaposi's sarcoma (according to ICD 10 - C46) than others. For example, more often this disease is observed in men than in women. The risk group primarily includes:
- HIV-infected. Kaposi's sarcoma is diagnosed very often in them.
- Men who are elderly and of Mediterranean race.
- People whose homeland is equatorial Africa.
- People who have transplanted organs from donors (especially kidneys).
Scientists agree that most often, especially in the initial stages, this disease is more of a reactive process (that occurs in response to infectious lesions) than a real sarcoma.
What are the possible complications of this disease?
The occurrence of complications of Kaposi's sarcoma depends primarily on the stage of development of this disease, and in addition, on the location of the tumors. The following complications are likely to occur:
- Suspension of motor activity of the limbs along with their deformation.
- The appearance of bleeding from decaying tumors.
- The onset of intoxicationorganism caused by the decay of neoplasms.
- Appearance of lymph edema.
- Presence of bacterial infection of damaged tumors.
- Failure of the internal organs in case of neoplasms on them.
Some complications can lead to life-threatening conditions for the patient. Next, consider what symptoms are observed in patients against the background of the development of this pathology.
General symptoms of pathology
Kaposi's sarcoma can be suspected if the first neoplasms appear on the surface of the skin. They usually have the following symptoms:
- The color of such a formation is bright burgundy, but it is also possible to acquire a blue-violet hue. I must say that when pressed, the color does not change. The spots may have a glossy surface, in addition, sometimes they are slightly flaky.
- The shape of the formation is flat and slightly rises above the skin surface. This disease is characterized by a multiplicity of rashes. In the case of fusion of spots, the center of the neoplasms sinks, turning into an ulcer. The symptoms of Kaposi's sarcoma don't end there.
- Neoplasms almost do not manifest themselves in the form of any sensations. Only as a result of merging into large foci can itching occur with tingling and swelling of the surrounding tissues.
- In the case of localization of a malignant tumor on the mucous membranes, signs are possible that depend on the specific sites of the lesion. So, there may be diarrhea along with vomiting, cough with bloody sputum, painduring chewing, swallowing and so on. The greatest danger is the development of a tumor in the digestive system or respiratory organs. This is how Kaposi's sarcoma manifests.
The course of the disease is usually slow, but progressive. If the spots are damaged, bleeding is likely, since the formations are predominantly a network of blood vessels. Symptoms of Kaposi's sarcoma depend on its subtype. In total, four typical varieties of this pathology are distinguished, we will consider them in more detail.
Classic type of this disease
How does he manifest himself? This type is typical for European countries. At the same time, tumor spots with clear boundaries are located on the feet and hands. Very rarely they are accompanied by itching with burning. This form of Kaposi's sarcoma goes through several stages:
- Spotty stage. This stage is characterized by the appearance of smooth spots that have jagged edges up to five millimeters in diameter.
- Papular stage. During this stage of Kaposi's sarcoma, the spots become like spheres, they thicken and increase in size up to one centimeter. In addition, they can merge and form a focus with a compacted shape and a rough surface.
- Tumor stage of Kaposi's sarcoma. This stage can be characterized by the formation of purple nodules that are relatively firm and ulcerate.
Endemic type of disease
This type is especially common among the population of Central Africa. Hediffers in the occurrence of such neoplasms in children under one year old, localized mainly on the tissues of organs and lymph nodes, practically without affecting the skin.
What kind of disease is Kaposi's sarcoma, not everyone knows.
Epidemic type of disease
It is associated with HIV infection. The epidemic type is considered sufficient for the diagnosis of AIDS, it can affect persons at a fairly young age up to forty years. The spots are bright red. Formations are localized on the nose, upper limbs and in the oral cavity, lesions of the mucous and lymph nodes, as well as internal organs, are necessarily present. This is the most rapid and life-threatening form of Kaposi's sarcoma.
Immunosuppressive type of pathology
This variety has a positive outlook. Most often, it occurs immediately after a donor kidney transplant due to the use of specific drugs, namely immunosuppressants. If these drugs are discontinued, the disease instantly regresses.
Among other things, Kaposi's sarcoma can occur in acute, subacute and chronic forms. Against the background of an acute course, a rapid progression of the pathology with an unfavorable outcome is observed. The course of the subacute type depends on the effectiveness of the therapeutic effect. The most favorable prognosis is observed in chronic forms of the disease, when the patient can do without treatment for more than ten years. The type of pathology is determined as a result of specific diagnostic measures, whichwill be discussed further.
Diagnosis of Kaposi's sarcoma should be timely.
Carrying out the diagnosis of pathology
Often an accurate diagnosis can be established without any research, because this disease almost always has vivid clinical manifestations. It is enough to carefully examine the skin and mucous membranes of the oral cavity, as well as the genital organs. It should be borne in mind that as a result of a biopsy, the discovery of bleeding is not excluded, since the tumor consists mainly of overgrown vessels. But if a specialist has doubts and in order to differentiate this pathology, the following diagnostic measures are prescribed:
- Thanks to the method of laboratory research, it is possible to detect the presence of the herpes virus of the eighth type and, accordingly, antibodies to it. This method will not indicate the development of Kaposi's sarcoma, but if the diagnosis is confirmed, it will help to more accurately determine the measures of drug exposure.
- It is mandatory for patients to be tested for the presence of HIV infection.
- Histological examination will help differentiate this pathology from fibrosarcoma, reflecting, among other things, the presence of a hemorrhagic component.
- In case of suspicion of localization of neoplasms in the respiratory tract or digestive system, a study of all these organs is prescribed by appropriate methods, for example, bronchoscopy, gastroscopy, colonoscopy, and so on.
- Probably the appointment of an immunogram to determine the status of cellular immunity, sincethis disease occurs due to a significant decrease in it.
- Additional measures may be needed in the form of an abdominal ultrasound. Also, computed tomography of the kidneys and adrenal glands is considered not superfluous. Thanks to this, the doctor will be able to get the most accurate picture of the damage to the internal organs.
Sometimes a doctor needs to differentiate Kaposi's sarcoma from other diseases that are similar in their manifestations. For example, this pathology must be differentiated from diseases such as lichen planus, mycosis fungoides, sarcoidosis, hemosiderosis, microvenular hemangioma, melanoma, and so on. The results of the diagnostics are used in the choice of therapeutic measures. Next, we will figure out how this pathology is treated.
Modern methods of treating the disease
Specific therapy for Kaposi's sarcoma is often not carried out. The impact on the tumor rarely gives any positive results. Basically, therapeutic procedures are directed to the disease that accompanies sarcoma, and in addition, to relieve symptoms and to alleviate the patient's well-being. They try to act directly on the tumor itself in the following cases:
- Very large tumor size.
- When the patient experiences severe pain and burning.
- In the case when the tumor is life threatening and is located on the mucous membranes of the respiratory tract or digestive system.
- When the tumor is a serious cosmetic defect.
In these cases, a local effect on the tumor can be applied, within which radiation therapy and cryotherapy are carried out along with the use of drugs in the form of Panretin, Prospidin and Dinitrochlorobenzene. All medicinal substances are injected into the tumor. In the event that the neoplasm is single, then a surgical operation is performed in the format of its excision. In addition, there may be a systemic impact, which is carried out in several directions at once:
- Chemotherapy has different side effects and requires combination with hormonal treatment. Patients typically require four courses per year.
- Carrying out antiretroviral therapy, which increases the immune status while suppressing the activity of the virus. This method sometimes gives good results.
- Treatment with interferons is carried out for long courses, it can have an immunomodulatory effect.
Topical treatment of Kaposi's sarcoma is performed on an outpatient basis and generally does not cause any side effects. It is prescribed for not very large tumors. The main danger of such treatment is the discovery of bleeding from the tumor, and in addition, the formation of ulcers on it, which can lead to infection, since the immunity of such a patient is very low. In addition, damaged tumors provoke the appearance of new lesions.
Systemic treatment is more often used when the patient does not experience severe symptoms and does not differ significantlyimmunodeficiency. But when the tumor is threatening or causes significant physical suffering, this treatment option is also used.
All methods of treatment that are used in relation to Kaposi's sarcoma, give only hope for recovery, but not a guarantee. Even a decrease in the size of the tumor along with its blanching or the absence of new formations are considered as positive results of treatment.
Prognosis for this severe pathology
The prognosis of Kaposi's sarcoma directly depends on its immediate form, course features and, of course, on the patient's immunity. Against the background of a sufficiently high immune status, a tumor-like formation can be reversible, and therapeutic measures, in turn, will give a positive result, allowing for a long-term remission.
In other situations, the forecast will be extremely unfavorable. More than fifty percent of patients die in extremely short terms from three months to two years. The faster the sarcoma progresses, the less chance there will be for a successful fight against it. Conversely, slow progression gives more chances to find the most effective method of influence.
A positive outcome of treatment for this disease does not increase the chances of a cure for AIDS. But for a patient with HIV infection, a complication in the form of the development of Kaposi's sarcoma can be fatal. This does not apply to immunosuppressive sarcoma, which has a benign course with oftenfavorable outcome.
Prevention of this disease
Preventive measures mainly reduce to the prevention of the disease of herpes of the eighth type. It also has an effect on immunodeficiency states and diseases that are accompanied by Kaposi's sarcoma.
The main condition for the prevention of this disease is the presence of a strong immune status. This also applies to cases where a disease has already arisen that can be accompanied by the occurrence of Kaposi's sarcoma. I must say that in HIV-infected patients, therapy with antiretroviral drugs gives good results and allows you to maintain an active immune system, preventing the manifestations of this pathology.
For patients whose disease is in remission, regular monitoring is required in the form of examination of the mucous membranes and skin once every three months, along with studies of the respiratory tract and digestive system. This will allow you to notice the recurrence of the disease in time.
Kaposi's sarcoma is often a life-threatening disease. Strong immunity acts as a natural and reliable protector of the body from most of all kinds of diseases. In this regard, it is extremely important to take care of supporting the defenses of your body, because only they can save a person from many serious life-threatening ailments.
