Brambilla Lucia, Genovese Giovanni, Berti Emilio, Peris Ketty, Rongioletti Franco, Micali Giuseppe, Ayala Fabio, Della Bella Silvia, Mancuso Roberta, Calzavara Pinton Piergiacomo, Tourlaki Athanasia
Unit of Dermatology, Maggiore Polyclinic Hospital, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Department of Medical-Surgical Pathophysiology and Transplantation, University of Milan, Milan, Italy.
Ital J Dermatol Venerol. 2021 Jun;156(3):356-365. doi: 10.23736/S2784-8671.20.06703-6. Epub 2020 Nov 12.
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.
卡波西肉瘤（KS）是一种与人类疱疹病毒8型（HHV8）感染相关的淋巴管增生性疾病。公认有四种临床亚型：经典型、地方性、流行性（与HIV相关）和医源性。KS的诊断基于临床特征、组织病理学评估和HHV8血清学检查。经典型KS通常局限于皮肤，病程呈慢性，而医源性变体可能表现为黏膜、淋巴结或内脏受累。临床分期对于指导治疗至关重要。局限性疾病即使没有比较不同治疗方式的随机试验，也可用不同的局部治疗方法进行治疗。侵袭性、播散性KS以及伴有内脏受累的病例通常需要全身化疗，最常用的是长春碱、博来霉素或紫杉醇。医源性KS需要逐渐减少/停用免疫抑制剂，对于移植后KS，如有可能，改用m-TOR抑制剂。由一组意大利专家撰写的本研究基于对文献的批判性回顾以及长期广泛的个人经验，提供了关于KS诊断和治疗的指南。
