Kaposha's sarcoma (multiple hemorrhagic idiopathic sarcoma of Kaposha, Kaposha's angiosarkom, angioendoteliom of skin) — multiple malignant defeats the terms developing from an endoteliya of the lymphatic and blood vessels passing in it. Often the disease is followed by defeat mucous a mouth and lymph nodes. Kaposha's sarcoma is shown by the multiple cyanotic and reddish spots on skin which are transformed to tumoral knots to 5 cm in the diameter. Diagnosis of sarcoma of Kaposha includes histology of a biopsiyny sample from the defeat center, a research of immunity and blood test on HIV. Treatment is carried out by system and local application of himiopreparat and interferon, beam impact on tumoral knots, cryotherapy.
Kaposha's sarcoma received the name by name the Hungarian dermatologist who for the first time described a disease in 1872. Prevalence of sarcoma of Kaposha is not so big, but among sick HIV it reaches 40-60%. At these patients Kaposha's sarcoma is the most often found malignant tumor. According to the clinical trials conducted in dermatology Kaposha's sarcoma is diagnosed for men approximately by 8 times more often than at women.
Treat risk groups in which development of sarcoma of Kaposha is most probable: the infected HIV of the man, the man of the Mediterranean origin at advanced age, nationality of the Central Africa, recipients of transplanted organs or patients, it is long receiving immunosupressivny therapy.
Reasons of sarcoma of Kaposha
The exact reasons of development of sarcoma of Kaposha and her pathogenesis are still unknown to medicine. The disease quite often arises against the background of other malignant processes: fungoid mycosis, Hodzhkin's disease, limfosarkoma, miyelomny disease. Researches of the last years confirm connection of a disease with presence at an organism of the specific antibodies which are formed at contamination a herpes virus 8 types. Infection with a virus can occur sexually, through blood or saliva.
Today scientists managed to allocate the tsitokina having the stimulating effect on cellular structures of sarcoma of Kaposha. Carry to them: 3FGF — a factor of growth of fibroblast, IL-6 — Interlaken 6, TGFp — the transforming growth factor. In developing of a tumor at patients with AIDS the great value is allocated for an onkostatin.
Kaposha's sarcoma is not absolutely usual malignant tumor. Weak mitotic activity, dependence of process on the immune status of the patient, multicentric development, a possibility of the return development of the centers of a tumor, lack of a cellular atipiya and histologic identification of signs of an inflammation confirm the assumption that at the beginning of the emergence Kaposha's sarcoma represents jet process rather, than true sarcoma.
Symptoms of sarcoma of Kaposha
Most often the disease begins with emergence on skin of violet-blue spots which infiltrirutsya with formation of roundish disks or knots. In some cases Kaposha's sarcoma begins with emergence on skin of papules that has similarity to rashes red flat depriving. Knots gradually grow, reaching size forest or walnut. They have the dense and elastic consistence and the shelled surface which is often penetrated by vascular asterisks. Knots hurt the patient, amplifying with a pressure upon them. Over time at Kaposha's sarcoma perhaps spontaneous rassasyvaniye of knots with formation of hems in the form of the pressed hyperpegmentations.
Allocate 4 clinical options of sarcoma of Kaposha: classical, epidemic, endemic and immune .
Classical sarcoma of Kaposha is widespread in Russia, Ukraine, Belarus and the countries of Central Europe. Its typical localization — skin of a side surface of shins, feet and brushes. The accurate ocherchennost and symmetry of the tumoral centers, lack of subjective symptomatology is characteristic (only in certain cases patients note burning or an itch). This form of sarcoma extremely seldom proceeds with defeat of mucous. During classical sarcoma of Kaposha allocate 3 clinical stages: spotty, papular and tumoral.
Spotty stage — the beginning of a disease. It is presented red or red-brown spots of an uneven form which size does not exceed 0,5 cm. Spots have a smooth surface.
The papular stage of sarcoma of Kaposha has an appearance of the multiple elements acting over skin level in the form of spheres or hemispheres. They are dense, elastic, diameter fluctuates from 0,2 to 1 cm. Merging among themselves, elements can form the semi-spherical or flattened plaques with a rough or smooth surface. But separate elements of sarcoma of Kaposha of this stage are more often remain isolated.
The tumoral stage is characterized by formation of tumoral knots with a diameter from 1 to 5 cm of Knots, as well as elements of a spotty stage, have a cyanotic-red or reddish-brown shade. They merge with each other and izjyazvlyatsya.
Epidemic sarcoma of Kaposha is associated with AIDS and is one of characteristic symptoms of this disease. The young age of the diseased (up to 37 years), brightness of vysypny elements and an unusual arrangement of tumors is typical: mucous, nose tip, top extremities and hard palate. At this form of sarcoma of Kaposha there is a bystry involvement in process of lymph nodes and internals.
Endemic sarcoma of Kaposha meets, as a rule, in the Central Africa. The disease develops at children's age, a thicket on the first year of life. It is characterized by damage of internals and lymph nodes, changes of skin are minimum and rare.
Immune Kaposha's sarcoma proceeds chronically and is most good-quality, as a rule, without involvement of internals. Arises against the background of the carried-out immunnosupressivny treatment at the patients who transferred organ transplantation. At cancellation of the medicines suppressing immunity often there is a regress of symptoms of a disease.
The course of sarcoma of Kaposha can be sharp, subsharp and chronic. At sharp development of a disease there is a bystry generalization of malignant process to development of intoxication and a kakheksiya. Patients perish during the period from 2 months to 2 years from the beginning of a disease. The subsharp form without performing treatment can last 2-3 years. Slow development of tumoral process at a chronic form of sarcoma of Kaposha brings to its longer current (8-10 and more years).
To complications of sarcoma of Kaposha deformation and restriction of movements in the affected extremities, bleedings from the breaking-up tumors, treat with development of a limfedema at a sdavleniye by a tumor of lymphatic vessels, etc. The ulceration of tumoral knots is dangerous by a possibility of infection that against the background of an immunodeficiency leads to bystry development of sepsis and can be the reason of a lethal outcome.
Diagnosis of sarcoma of Kaposha
Diagnosis of sarcoma of Kaposha is based on its typical clinical manifestations and educated a biopsy. The histologic research reveals in a term multiple neogenic vessels and proliferation of spindle-shaped cages — young fibroblast. Existence of a gemosiderin and ekstravazat (hemorrhagic exudates) allows to distinguish Kaposha's sarcoma from a fibrosarkoma.
To patients conduct immunological blood tests and the analysis on HIV. At Kaposha's sarcoma apply to identification of damages of internals ultrasonography of an abdominal cavity and heart, gastroscopy, a X-ray analysis of lungs, a stsintigrafiya of a skeleton, KT of kidneys, MRT of adrenal glands, etc.
Differential diagnosis of sarcoma of Kaposha with red flat is necessary it is deprived, sarkoidozy, gemosiderozy, by fungoid mycosis, high-differentiated angiosarkomy, mikrovenulyarny gemangiomy, etc.
Treatment of sarcoma of Kaposha
Important point in treatment of sarcoma of Kaposha is therapy of the main disease for increase in the immune status of an organism. System treatment of sarcoma of Kaposha is usually appointed to patients with a favorable immunological background at an asymptomatic course of disease. It consists in intensive polychemotherapy. The risk of application of similar therapy for patients with an immunodeficiency is connected with toxic impact of medicines on marrow, especially in combination with medicines for treatment of HIV infection. On 1 and 2 stages of sarcoma of Kaposha of the most optimum the unicomponent chemotherapy prospidiy is considered chloride which differs in lack of the oppressing action on immune system and blood formation. In treatment of a classical and epidemic form of sarcoma of Kaposha interferona are successfully applied: α-2a, α-2b and β. They interfere with proliferation of fibroblast and are capable to initiate apoptosis of tumor cells.
Local treatment of sarcoma of Kaposha the chloride prospidiya, other methods includes cryotherapy, injections of chemotherapeutic medicines and interferon in a tumor, applications of dinitrobenzene chloride and ointment. At existence of the painful and large centers of defeat, and also for the best cosmetic effect local radiation is carried out.
Forecast of sarcoma of Kaposha
The forecast of a disease at Kaposha's sarcoma depends on character of its current and is closely connected with a condition of immune system of the patient. At higher rates of immunity of display of a disease can have reversible character, system treatment gives good effect and allows to achieve remission from 50-70% of patients. So at patients with Kaposha's sarcoma with an indicator of lymphocytes of CD4 more than 400 mkl-1 the frequency of remissions against the background of the carried-out immune therapy exceeds 45%, and at CD4 less than 200 mkl-1 it is possible to achieve remission only 7% of patients.