S1 Guidelines for the Kaposi Sarcoma

Abstract

Summary Kaposi’s sarcoma (KS) is a rare, malignant, multilocular vascular disease originating from lymphatic endothelial cells that can primarily affect the skin and mucous membranes, but also the lymphatic system and internal organs such as the gastrointestinal tract, lungs or liver. Five epidemiological subtypes of KS with variable clinical course and prognosis are distinguished, with increased incidence in specific populations: (1) Classical KS, (2) Iatrogenic KS in immunosuppression, (3) Endemic (African) lymphadenopathic KS, (4) Epidemic, HIV‐associated KS and KS associated with immune reconstitution inflammatory syndrome (IRIS), and (5) KS in men who have sex with men (MSM) without HIV infection. This interdisciplinary guideline summarizes current practice‐relevant recommendations on diangostics and therapy of the different forms of KS. The recommendations mentioned in this short guideline are elaborated in more detail in the extended version of the guideline (online format of the JDDG).

Case Series of Kaposi Sarcoma in HIV-Negative MSM

“…This subtype usually occurs in young or middle-aged males who have sex with other men. It has a clinical presentation similar to classic KS with usually an indolent course and extension limited to the skin, although lymph node involvement has been reported in 5% of patients [7]. No immune deficiencies are present [2][3][4][5][6].…”

Unusual Localization of AIDS-Related Kaposi’s Sarcoma in a Heterosexual Male during the COVID-19 Pandemic: A Case Report

“…The risk of KS in people with AIDS is 30–80 times higher than in the general population, although the incidence has decreased in recent years, as effective antiretroviral therapy is used and persistent viral HIV replication is suppressed [ 10 , 11 ].…”

“…The patient developed a high-risk systemic form of the disease, evaluated according to the severity criteria proposed by the management guidelines for KS: an association of edemas, extensions of oral and visceral lesions, and immunosuppression with a CD 4 lymphocyte count below 200/mm 3 [ 11 ].…”

“…Ophthalmic localization of KS is rare. Until the beginning of the AIDS pandemic, only 25 cases of ophthalmic KS were reported in the US [ 11 ].…”

“…During the latent phase, the expression of genes associated with HHV8 is limited, but epigenetic factors can switch between the latent and the lytic form. Reactivation of the latent form can be caused by environmental factors, chemical agents, or hydrogen peroxide from reactive oxygen species [ 11 , 12 ].…”