Kaposi's sarcoma is a species of many malignant neoplasms that appear on the skin. This pathology was first described by the Hungarian dermatologist Moritz Kaposi, whose name today bears the name of this disease. Due to the specific appearance of neoplasms, this pathology is sometimes called hemorrhagic sarcoma.
I must say that the location of the tumors is not limited only to the skin, often they affect the internal organs along with the mucous membranes and lymph nodes. Next, we 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 performed.
Causes of the disease
Kaposi’s sarcoma is not very common among the population, but among HIV-infected people, especially in men, it manifests itself in half the cases. For certain, the causes that cause the appearance of such neoplasms are not known today. But scientists confidently suggest that this disease can develop on the basis of the eighth type of herpes virus, which is still not well understood. Quite often, pathology can accompany other malignant processes in the form of mushroom mycosis, 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, in some categories of people, the risk of Kaposi's sarcoma (ICD 10 - C46) is much higher than in others. For example, this disease is more often 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 belong to the Mediterranean race.
- People whose homeland is equatorial Africa.
- People who have organs transplanted from donors (especially kidneys).
Scientists agree that most often, especially in the initial stages, this disease is more a reactive process (which 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, first of all, 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 intoxication of the body caused by the breakdown of tumors.
- The appearance of lymphatic edema.
- The presence of bacterial infection of damaged tumors.
- Failure of the internal organs in case of presence of neoplasms on them.
Some complications can lead to life-threatening conditions for the patient. Next, we consider what symptoms are observed in patients against the background of the development of this pathology.
General symptoms of pathology
The development of Kaposi’s sarcoma can be suspected in the event of the appearance of the first neoplasms on the surface of the skin. Typically, they are characterized by the following symptoms:
- The color of this formation has a bright burgundy, but it is possible to acquire a blue-violet hue. I must say that when pressed, the color does not change. Spots can have a glossy surface, in addition, sometimes they peel slightly.
- The shape of the formation is flat and rises slightly above the skin surface. This disease is characterized by a multiplicity of rashes. In the case of the confluence of spots, the neoplasm center sinks, turning into an ulcer. The symptoms of Kaposi’s sarcoma do not end there.
- Neoplasms almost do not manifest themselves in the form of any sensations. Only as a result of fusion into large foci can itching occur with tingling and swelling of the surrounding tissues.
- In the case of localization of a malignant formation on the mucous membranes, signs are possible that depend on the specific lesion sites. So, diarrhea can be observed along with vomiting, cough with bloody sputum, pain during chewing, swallowing, and so on. The greatest danger is the development of a tumor in the digestive system or respiratory system. This is how Kaposi’s sarcoma appears.
The course of the disease is usually slow, but progressive. If the spots are damaged, bleeding is likely, since the formations are mainly a network of 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.
The 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. It is extremely rare that they are accompanied by itching with burning. This form of Kaposi’s sarcoma goes through several stages:
- Spotted stage. This stage is characterized by the appearance of smooth spots, which have uneven edges with a diameter of up to five millimeters.
- Papular stage. During this stage of Kaposi’s sarcoma, the spots become like spheres, they become denser and increase in size 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 nodes with a purple hue, which are relatively dense and ulcerate.
Endemic type of disease
This type is especially prevalent among the population of Central Africa. It is distinguished by the occurrence of such neoplasms in children up to a 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 people at a fairly young age up to forty years. The spots are bright red. Localized formations on the nose, upper limbs and in the oral cavity, without fail, there are lesions of the mucous and lymph nodes, and in addition, internal organs. This is the most fleeting and life-threatening form of Kaposi’s sarcoma.
Immunosuppressive type of pathology
This variety has a positive prognosis. Most often, it occurs immediately after a donor kidney transplant due to the use of specific drugs, namely immunosuppressants. If these drugs are canceled, the disease instantly regresses.
Among other things, Kaposi’s sarcoma can occur in acute, subacute and chronic form. Against the background of the acute course, rapid progress of 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 medical methods for more than ten years. The type of pathology is determined as a result of specific diagnostic measures, which will be discussed later.
Diagnosis of Kaposi's sarcoma should be timely.
Diagnosis of pathology
Often, an accurate diagnosis can be made 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 genitals. It should be borne in mind that as a result of a biopsy, the opening of bleeding is not excluded, since the tumor consists mainly of overgrown vessels. But if a specialist doubts and in order to differentiate this pathology, the following diagnostic measures are prescribed:
- Thanks to the method of laboratory research, the presence of the herpes virus of the eighth type and, accordingly, antibodies to it can be detected. 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.
- Without fail, patients are prescribed tests for the presence of HIV infection.
- A histological examination will help to differentiate this pathology from fibrosarcoma, reflecting, among other things, the presence of a hemorrhagic component.
- In case of suspicion of the localization of neoplasms in the area of the respiratory tract or digestive system, a study of all these organs is prescribed by suitable methods, for example, bronchoscopy, gastroscopy, colonoscopy and so on are performed.
- It is likely that an immunogram is prescribed to determine the status of cellular immunity, since this disease occurs due to its significant decrease.
- Additional measures may be necessary in the form of an ultrasound examination of the abdominal region. Also considered computed tomography of the kidneys and adrenal glands is 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 differentiation of Kaposi’s sarcoma from other diseases similar in their manifestations. For example, this pathology should be differentiated from such ailments as lichen planus, fungoid mycosis, sarcoidosis, hemosiderosis, microvenous hemangioma, melanoma, and so on. The results of the diagnosis are used when choosing treatment measures. Next, we will understand how the treatment of this pathology is carried out.
Modern methods of treating the disease
Kaposi’s sarcoma specific therapy is often not performed. Exposure to the tumor extremely rarely gives any positive results. In general, therapeutic procedures are directed to the disease that accompanies sarcoma, and in addition, to relieve symptoms and to make the patient better. They try to directly influence the tumor itself in the following cases:
- Very large tumor size.
- When the patient experiences severe pain and burning sensation.
- In the event that the tumor is life threatening and is located on the mucous membranes of the respiratory tract or digestive system.
- When a tumor is a serious cosmetic defect.
In these cases, a local effect on the tumor can be applied, within the framework of which radiation therapy and cryotherapy are carried out along with the use of drugs in the form of Panretin, Prospidin, and Dinitrochlorobenzene. All drugs 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, a systemic impact may be exerted, which is carried out immediately in several directions:
- Chemotherapy is characterized by side effects and requires a combination with hormonal treatment. Patients usually require four courses per year.
- Conducting antiretroviral therapy, which enhances the immune status while suppressing the activity of the virus. This method sometimes gives high results.
- Treatment with interferons is carried out in long courses, it can have an immunomodulatory effect.
Local treatment of Kaposi’s sarcoma is performed on an outpatient basis, basically it 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 foci.
Systemic treatment is often used when the patient does not experience severe symptoms and does not differ in significant immunodeficiency. But when the tumor is threatening or causes significant physical suffering, a similar treatment option is also used.
All treatment methods that are used in relation to Kaposi’s sarcoma give only hope for recovery, but not a guarantee. As a positive result of treatment, even a decrease in the tumor in size along with its blanching or the absence of new formations is considered.
The prognosis for this severe pathology
The prognosis of Kaposi’s sarcoma directly depends on its immediate form, the characteristics of the course and, of course, on the patient’s immunity. Against the background of a sufficiently high immune status, the tumor formation can be reversible, and therapeutic measures, in turn, will give a positive result, allowing for a long-term remission.
In other situations, the prognosis 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 likely it will be to successfully fight it. Conversely, slow progression is more likely to find the most effective mode of exposure.
A positive 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 developing Kaposi’s sarcoma can be fatal. This does not apply to immunosuppressive sarcoma, which has a benign course with an often favorable outcome.
Prevention of this disease
Preventive measures mainly reduce the prevention of herpes simplex disease of the eighth type. It also affects immunodeficiency conditions 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 when a disease has already occurred that can be accompanied by the onset of Kaposi's sarcoma. I must say that in HIV-infected patients, antiretroviral therapy gives good results and allows you to maintain immunity in an active form, preventing the manifestations of this pathology.
For patients whose disease is in remission, regular monitoring is required in the form of an examination of the mucous membranes and skin every three months, along with studies of the respiratory tract and digestive system. This will allow you to notice a relapse of the disease in time.
Kaposi's sarcoma is often a life-threatening disease. Strong immunity acts as a natural and reliable defender of the body against most of all kinds of diseases. In this regard, it is extremely important to take care of the support of the defenses of your body, because only they can protect a person from many serious ailments that threaten life.