Kaposha’s sarcoma – the reasons, symptoms, types, diagnostics and treatment of sarcoma of Kaposha

What is Kaposha’s sarcoma?

 Kaposha's sarcoma – the reasons, symptoms, types, diagnostics and treatment of sarcoma of Kaposha

Kaposha’s sarcoma – a type of multiple malignant new growths on skin. For the first time the disease was described in works of the Hungarian dermatologist Moritz Kaposcha on behalf of whom received the name.

Because of appearance of new growths the disease is called sometimes hemorrhagic sarcoma of Kaposha. Localization of tumors is not limited to an integument, often affecting an internal, mucous membranes and lymph nodes.

Reasons of development of sarcoma of Kaposha

This disease is not really widespread among the population, but at HIV-positive people, especially men, is shown almost in half of cases.

For certain the reasons causing emergence of such new growths are not known. But scientists with high probability assume that the disease can develop against a virus of herpes of the person of the 8th type which in itself is still insufficiently studied.

Also often Kaposha’s sarcoma accompanies other malignant processes, among which:

  • fungoid mycosis

  • Hodzhkin’s lymphoma (limfogranulematoz)

  • limfosarkoma

  • multiple myeloma

  • leukoses

Developing of pathology requires considerable decrease in immunity of the person owing to the different reasons. Besides, at some groups of people the risk of emergence of sarcoma of Kaposha is much higher, than at the others. So, for example, more often the illness is observed at men, than at women.

Risk are in group:

  • HIV-positive persons

  • the men of advanced age belonging to the Mediterranean race

  • persons which homeland is the equatorial Africa

  • persons with the bodies replaced from donors (especially a kidney)

Scientists-physicians are unanimous in one: most often, especially in initial stages of development, this disease represents jet process (that is arising in response to infectious defeat), than true sarcoma rather.

Possible complications of a disease

Emergence of complications of sarcoma of Kaposha depends on a stage of development of a disease and places of localization of tumors. Emergence of the following complications is possible:

  • restriction of physical activity of extremities and their deformation

  • bleedings from the breaking-up tumors

  • the organism intoxication caused by disintegration of new growths

  • lymphatic hypostases, slonovost owing to displacement of lymph nodes

  • bacterial infection of the damaged tumors

  • violation of work of an internal at localization of new growths on them

Some complications lead to the states menacing to human life.

General symptoms of sarcoma of Kaposha

 Kaposha's sarcoma – the reasons, symptoms, types, diagnostics and treatment of sarcoma of Kaposha

It is possible to suspect development of sarcoma of Kaposha at emergence of the first new growths on skin. Usually for them it is characteristic:

  • Color – is more often bright claret, but is possible from red to blue-violet (on this sign it is similar to bruise), when pressing color does not change. Spots have a glossy surface, sometimes are slightly shelled.

  • Form – in the form of a spot, the flat or slightly towering over a surface skin. Plurality of rashes is characteristic. At merge of spots the center of a new growth can sink down and turn into an ulcer.

  • Feelings – new growths practically do not prove this sign. Only at merge in the big centers there can be an itch, a pricking, hypostasis of surrounding fabrics.

  • At localization of malignancies on mucous membranes the signs depending on a concrete place of defeat (diarrhea, vomiting with blood, cough with a bloody phlegm, pain are possible at a chewing and swallowing of food). Big hazard to life is posed by development of tumors in a digestive tract and respiratory organs.

Course of disease slow, but steadily progressing. At damage of spots bleedings as they generally represent a network of vessels are possible. The symptomatology of a disease depends on its subtype.

Standard kinds of sarcoma of Kaposha

Kaposha’s sarcoma can proceed on one of the types classified by physicians depending on symptomatology, the territory of distribution and to the forecast.

Allocate 4 standard kinds of this disease:

Classical type

It is characteristic for the European countries. Tumor spots with a clear boundary are localized on feet, shins, brushes. Seldom are followed by an itch or burning.

The disease flowing on this type passes some stages:

  • spotty (it is characterized by emergence of deckle-edged smooth spots diameter to

    5 mm)

  • papular (spots get a sphere form, become dense naoshchup, increase in the amount up to

    1 cm, can merge, forming the center of the flattened form with a rough surface)

  • tumoral (it is characterized by formation of knots of a violet shade which can be more or less dense, izjyazvlyatsya)

Endemichesky type

It is widespread among inhabitants of the Central Africa. Differs in emergence at infantile age (at children till 1 year), localization on fabrics of an internal and lymph nodes, practical lack of damages of skin.

Epidemic type

Is associated with HIV infection. AIDS is sufficient for statement of the diagnosis. Strikes persons of quite young age (till 40 years). Spots of bright red color, localization place: the nose, the top extremities, the top sky in an oral cavity, surely are present damages of mucous membranes, lymph nodes and internal. It is the most rapid and zhizneugrozhayushchy form of sarcoma of Kaposha.

Immunnosupressivny type

Has the positive forecast. Most often arises after change of a donor kidney owing to reception of specific medicines – immunosupressor. At cancellation of preparations the disease regresses.

Besides, the disease can proceed in sharp, subsharp and chronic forms. At a sharp current the rapid progress and a frequent failure is observed. The current of a subsharp form depends on efficiency of medical influence. The most favorable forecast is observed at chronic forms of an illness when the patient can do without medical measures more than 10 years.

The type of sarcoma of Kaposha is defined as a result of diagnostic actions.

Diagnosis of a disease

 Kaposha's sarcoma – the reasons, symptoms, types, diagnostics and treatment of sarcoma of Kaposha

Often the exact diagnosis at Kaposha’s sarcoma can be established and without various researches, this disease has so bright clinical manifestations. Rather attentive survey by the expert of integuments, mucous membranes of a mouth and genitals. Besides as a result of a biopsy bleeding as the tumor consists of the expanded vessels can open.

But at emergence at the expert of doubts and for differentiation of this disease diagnostic actions are appointed:

  • By method of laboratory researches (PTsR) it is possible to reveal presence at an organism of a virus of herpes of the 8th type and anti
    bodies to it. This way authentically will not indicate existence of sarcoma of Kaposha or possibility of its development, but at confirmation of the diagnosis will help to appoint more precisely measures of medicamentous influence.

  • Without fail to patients the analysis on existence of HIV infection is carried out.

  • Histologic research will help to differentiate this disease from a fibrosarkoma, having shown among other existence of a hemorrhagic component.

  • At suspicion research of these systems is appointed in the suitable ways to localization of new growths in airways, a digestive tract (about what can tell complaints of the patient) (a bronkhoskopiya or a X-ray analysis of lungs, gastroscopy and a kolonoskopiya – for inspection of digestive organs).

  • Purpose of an immunogramma with definition of the status of cellular immunity as the disease arises against its considerable decrease is possible.

  • Additional actions in the form of ultrasonic research of an abdominal cavity, a computer tomography of kidneys, MRT of adrenal glands can be demanded, a stsintigrafiya of bones. Thus the doctor can receive the fullest picture of defeats of an internal.

Differentiation of sarcoma of Kaposha from diseases, similar on manifestations, can sometimes be necessary for the doctor: red flat depriving, fungoid mycosis, a sarkoidoz, a gemosideroz, a mikrovenulyarny gemangioma, a melanoma, etc.

Results of diagnostics are used at a choice of therapeutic measures.

Treatment of sarcoma of Kaposha

 Kaposha's sarcoma – the reasons, symptoms, types, diagnostics and treatment of sarcoma of Kaposha

Specific treatment of sarcoma of Kaposha often is not carried out. Impact directly on a tumor seldom yields positive results. Generally therapeutic measures are directed on a disease to which sarcoma, and on removal of symptoms and relief of the general health of the patient accompanies.

Directly influence a tumor in cases:

  • very large size of a tumor

  • if the patient feels pain, strong burning

  • if the tumor threatens the patient’s life, settling down on mucous membranes of airways or a digestive tract

  • the tumor is serious cosmetic defect

In these cases impact on a tumor can be two types:

  1. Local influence – application of radiation therapy, cryotherapy, local putting medicines (Panretin, Prospidin, Dinitrokhlorbenzol), introduction of medicinal substances directly in a tumor. If the tumor is single, perform a surgery – its excision. About 40% of such operations have consequences as long remission so it is possible to call a method quite productive.

  2. System influence which, in turn, is conducted in several directions:

    • the chemotherapy – has side effects, sometimes demands a combination to hormonal therapy, it is necessary to carry out to 4kh courses in a year

    • anti-retrovirus therapy – promotes increase of the immune status with simultaneous suppression of virus activity, quite long influence (not less than a year), but sometimes yields good results

    • therapy of an interferonama – is also carried out by long courses, has immunomodulatory effect

Local therapy is carried out on an outpatient basis, generally without causing side effects. It is shown at not really big and single tumors. The main danger of treatment is discovery of bleeding from a tumor or formation on it of ulcers that can lead to infection as the immune status is very low. Besides, the damaged tumor provokes emergence of the new centers.

System influence is more often applied if the patient does not test the expressed symptoms and has no considerable immunodeficiency. But in cases when the tumor has the character menacing to life or causes physical sufferings, such option of treatment is also applied.

All methods of treatment applied relatively Kaposha’s sarcomas give only hope for recovery, but not a guarantee. As positive result of treatment reduction of a tumor in sizes, its pobledneniye or lack of new educations is considered even.

The forecast at Kaposha’s sarcoma

The forecast of this disease depends on its form, features of a current and a condition of immunity of the patient. At rather high immune status opukholevidny educations can have reversible character, medical measures yield positive result, allowing to achieve long remission.

In other cases forecast the extremely adverse. More than 50% of patients die in short terms (of 2-3 months to 2kh years). The quicker sarcoma progresses, the it is less than chances of successful fight against it. To the contrary, the slow current gives more opportunities to pick up an effective way of influence.

The positive result of treatment of sarcoma of Kaposha does not increase chances of recovery from AIDS. But for the patient with AIDS complication in the form of Kaposha’s sarcoma can become deadly.

It does not concern the facts of immunnosupressivny sarcoma which have a good-quality current and, in most cases, a favorable outcome.

Prevention of sarcoma of Kaposha

 Kaposha's sarcoma – the reasons, symptoms, types, diagnostics and treatment of sarcoma of Kaposha

Preventive measures, are generally reduced to prevention of a disease of herpes of the 8th type, immunodeficiency and diseases which can be followed by Kaposha’s sarcoma.

The basic in prevention of this disease – the strong immune status. It concerns also cases when already there was a disease which can be followed by emergence of sarcoma of Kaposha. So, at HIV-positive patients therapy by anti-retrovirus preparations yields good results, allowing to support immune system in an active form and to interfere with manifestations of sarcoma of Kaposha.

For patients which disease is in a condition of remission, regular control in the form of survey of mucous membranes and an integument (1 times in 3 months), researches of airways and a digestive tract is necessary (at least 1 time a half-year). It will give the chance in time to notice recurrence of a disease.

Kaposha’s sarcoma in many cases – the disease menacing to life. Strong immunity – the natural and reliable defender of an organism from many diseases. Therefore it is necessary to care of maintenance of protective forces of an organism, and they, in turn, are capable to save you from many problems.

Author of article: Bulls Evgeny Pavlovich, oncologist