Kaposi's sarcoma (KS) is a multifocal neoplasm of lymphatic endothelium-derived cells infected with human herpesvirus 8. Four clinical subtypes are distinguished: the classic, the endemic, the epidemic subtype in HIV positive patients and the iatrogenic subtype. The diagnosis is primarily based on clinical features and confirmation by histology with immunohistochemistry. Cutaneous distribution and severity, mucosal, nodal and visceral involvement depend on the type of KS with in general indolent behaviour and chronic evolution in the classic subtype and the more severe forms in iatrogenic or epidemic subtypes. Management should aim at achieving disease control. For localised lesions, several local therapies have been developed without randomised trial comparisons. Radiotherapy, intralesional chemotherapies and electrochemotherapy have high response rates. Topical treatments-imiquimod or topical 9-cis-retinoid acid-can also be used. Systemic treatments are reserved for locally aggressive extensive and disseminated KS: the recommended first-line agents are pegylated liposomal doxorubicin (PLD) and paclitaxel. In CKS, PLD or low-dose interferon-alfa are the recommended first-line agents in younger patients. In AIDS-related KS, combination antiretroviral therapy is the first treatment option; specific systemic treatment is needed only in case of extensive disease and in the prevention and treatment of immune reconstitution inflammatory syndrome. In post-transplant KS, tapering down immunosuppressive therapy and switching to mammalian target of rapamycin (m-TOR) inhibitors are used. Follow-up schedules for patients with KS disease depend on aggressiveness of the disease.

1st Department of Dermatology Venereology National and Kapodistrian University of Athens A Sygros Hospital Athens Greece

APHP Department of Dermatology INSERM U976 University Paris 7 Diderot Saint Louis University Hospital Paris France

APHP Department of Pathology INSERM U976 University Paris 7 Diderot Saint Louis University Hospital Paris France

Carol Davila University of Medicine and Pharmacy Bucharest Romania

Centre of Dermatovenereology Medical Science Institute Medical Faculty of Vilnius University Vilnius Lithuania

Department of Dermato oncology Université Paul Sabatier Institut Universitaire du Cancer de Toulouse Oncopole et CHU Larrey Toulouse France

Department of Dermatology Aix Marseille University Hôpital de la Timone Assistance Publique Hôpitaux de Marseille Marseille France

Department of Dermatology and Allergology University of Szeged Hungary

Department of Dermatology Coimbra University Hospital Centre Coimbra Portugal

Department of Dermatology Faculty of Medicine Military Medical Academy Belgrade Serbia

Department of Dermatology Hospital Clinic of Barcelona IDIBAPS and CIBER de raras Spain

Department of Dermatology Medical University of Vienna Austria

Department of Dermatology Royal London Hospital and Centre for Cutaneous Research Blizard Institute London United Kingdom

Department of Dermatovenerology 3rd Faculty of Medicine Charles University of Prague Prague Czech Republic

Department of Medical Oncology Gustave Roussy Comprehensive Cancer Center Villejuif Paris Sud France

Department of Oncology Oxford National Institute for Health Research Biomedical Research Centre United Kingdom

Department of Radiation Oncology Gustave Roussy INSERM U1030 Université Paris Saclay F 94805 Villejuif France

Dermatology Clinic University of Trieste Hospital Maggiore Piazza dell' Ospedale 1 34125 Trieste Italy

Institute of Dermatology Fondazione Policlinico Universitario A Gemelli IRCCS Catholic University Rome Italy

Sorbonne Université INSERM Institut Pierre Louis d'Epidémiologie et de Santé Publique AP HP Hôpital Pitié Salpêtrière Service d'Oncologie Médicale Paris France

University Department of Dermatology Erasme Hospital Universite´ Libre de Bruxelles Brussels Belgium

University Department of Dermatology Tuebingen Germany

University Hospital Zurich Department of Dermatology Zürich Switzerland

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