Bei starker Immunsuppression kommt es oft zu einem disseminierten, multifokalen Auftreten, ansonsten sind eher einzelne Manifestationen zu beobachten. Die Erkrankung greift hier oft direkt die inneren Organe an und zeigt keine Symptome der Haut. Mallery, P. Pei, D. Landwehr, C.
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They are typically found on the skin, but spread elsewhere is common, especially the mouth, gastrointestinal tract and respiratory tract. Growth can range from very slow to explosively fast, and is associated with significant mortality and morbidity. Red to brownish irregularly shaped macules and plaques. The lesions are usually as described above, but may occasionally be plaque -like often on the soles of the feet or even involved in skin breakdown with resulting fungating lesions.
Associated swelling may be from either local inflammation or lymphoedema obstruction of local lymphatic vessels by the lesion. Skin lesions may be quite disfiguring for the sufferer, and a cause of much psychosocial pathology.
In the mouth, the hard palate is most frequently affected, followed by the gums. Gastrointestinal tract[ edit ] Involvement can be common in those with transplant-related or AIDS-related KS, and it may occur in the absence of skin involvement.
Thus, kissing is a theoretical risk factor for transmission. Higher rates of transmission among gay and bisexual men have been attributed to "deep kissing" sexual partners with KSHV. Prudent advice is to use commercial lubricants when needed and avoid deep kissing with partners with KSHV infection or whose status is unknown.
KSHV is also transmissible via organ transplantation  and blood transfusion. Pathology[ edit ] Micrograph of a Kaposi sarcoma showing the characteristic spindle cells, high vascularity and intracellular hyaline globs. Despite its name, in general it is not considered a true sarcoma ,   which is a tumor arising from mesenchymal tissue. The histogenesis of KS remains controversial. KS lesions contain tumor cells with a characteristic abnormal elongated shape, called spindle cells.
The most typical feature of Kaposi sarcoma is the presence of spindle cells forming slits containing red blood cells. Mitotic activity is only moderate and pleomorphism is usually absent. Inflammation around the tumor may produce swelling and pain. Variously sized PAS positive hyaline bodies are often seen in the cytoplasm or sometimes extracellularly.
The spindle cells of Kaposi sarcoma differentiate toward endothelial cells , probably of lymph vessel rather than blood vessel nature. Dilated irregular vascular channels surround a pre-existing vessel. There is erythrocyte extravasation and hemosiderin pigmentation. Well-circumscribed spindle-cell tumor.
Erythrocytes lie within poorly defined slit-like vascular spaces. In differential diagnosis, arteriovenous malformations , pyogenic granuloma and other vascular proliferations can be microscopically confused with KS.
Since Moritz Kaposi first described this cancer, the disease has been reported in five separate clinical settings, with different presentations, epidemiology, and prognoses. Classic Kaposi sarcoma most commonly appears early on the toes and soles as reddish, violaceous, or bluish-black macules and patches that spread and coalesce to form nodules or plaques.
In most cases the treatment involves surgical removal of the lesion. The condition tends to be indolent and chronic, affecting elderly men from the Mediterranean region, Arabian countries  or of Eastern European descent.
Although this may be present worldwide, it has been originally described later in young African people, mainly from sub-Saharan Africa. This variant is not related to HIV infection   and is a more aggressive disease that infiltrates the skin extensively.
The tumor arises either when an HHV 8-infected organ is transplanted into someone who has not been exposed to the virus or when the transplant recipient already harbors pre-existing HHV 8 infection. In more advanced cases, they can be found in the stomach and intestines, the lymph nodes, and the lungs. However, these tests are not available except as research tools, and, thus, there is little screening for persons at risk for becoming infected with KSHV, such as people following a transplant.
Treatment[ edit ] Kaposi sarcoma is not curable, but it can often be treatable for many years. In KS associated with immunodeficiency or immunosuppression, treating the cause of the immune system dysfunction can slow or stop the progression of KS. People with a few local lesions can often be treated with local measures such as radiation therapy or cryosurgery.
In general, more widespread disease, or disease affecting internal organs, is treated with systemic therapy with interferon alpha, liposomal anthracyclines such as liposomal doxorubicin or daunorubicin , thalidomide , or paclitaxel. Likewise, no systematic screening of organ donations is in place. In people with AIDS, Kaposi sarcoma is considered an opportunistic infection , a disease that is able to gain a foothold in the body because the immune system has been weakened.
Because of their highly visible nature, external lesions are sometimes the presenting symptom of AIDS. Kaposi sarcoma entered the awareness of the general public with the release of the film Philadelphia , in which the main character was fired after his employers found out he was HIV-positive due to visible lesions. By the time KS lesions appear, it is likely that the immune system has already been severely weakened.
National Cancer Institute.