Giornale Italiano di Dermatologia e Venereologia 2020 Nov 12
Copyright © 2020 EDIZIONI MINERVA MEDICA
Italian guidelines for the diagnosis and treatment of classic and iatrogenic Kaposi’s sarcoma
Lucia BRAMBILLA 1, Giovanni GENOVESE 1, 2, Emilio BERTI 1, 2, Ketty PERIS 3, 4, Franco RONGIOLETTI 5, Giuseppe MICALI 6, Fabio AYALA 7, Silvia DELLA BELLA 8, 9, Roberta MANCUSO 10, Piergiacomo CALZAVARA PINTON 11, Athanasia TOURLAKI 1 ?
1 U.O. Dermatologia, Fondazione IRCCS Ca’ Granda - Ospedale Maggiore Policlinico, Milano, Italy; 2 Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Milano, Italy; 3 Dermatologia, Fondazione Policlinico Universitario A. Gemelli IRCCS, Roma, Italy; 4 Dermatologia, Università Cattolica del Sacro Cuore, Roma, Italy; 5 Unit of Dermatology, Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy; 6 Dermatology Clinic, University of Catania, Catania, Italy; 7 Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy; 8 Unit of Clinical and Experimental Immunology, Humanitas Clinical and Research Center-IRCCS, Rozzano, Milano, Italy; 9 Department of Medical Biotechnologies and Translational Medicine, University of Milan, Milan, Italy; 10 IRCCS Fondazione Don Carlo Gnocchi, Milano, Italy; 11 Department of Dermatology, University of Brescia, Brescia, Italy
Kaposi’s sarcoma (KS) is a lymphangioproliferative disorder associated with human herpesvirus 8 (HHV8) infection. Four clinical subtypes are recognized: classic, endemic, epidemic (HIV-related) and iatrogenic. KS diagnosis is based on clinical features, histopathological assessment, and HHV8 serology. Classic KS is usually skin-limited and has a chronic course, while the iatrogenic variant may show mucosal, nodal or visceral involvement. Clinical staging is fundamental to guide the management. Localized disease may be treated with different local therapies, even if there are no randomized trials comparing these different modalities. Aggressive, disseminated KS and cases with visceral involvement usually require systemic chemotherapy, most commonly vinblastine, bleomycin or paclitaxel. Iatrogenic KS needs immunosuppression tapering/withdrawal and, if possible, switch to m-TOR inhibitors in post-transplant KS. The present work by a panel of Italian experts provides guidelines on KS diagnosis and management based on a critical review of the literature and a long and extensive personal experience.
KEY WORDS: Kaposi’s sarcoma; HHV8; Guidelines; Management; Diagnosis