Diagnosis and treatment of Kaposi's sarcoma: European consensus-based interdisciplinary guideline (EDF/EADO/EORTC)

Highlights

• Kaposi's sarcoma (KS) is a human herpesvirus-8–associated multicentric lymph endothelial proliferation.

• KS severity depends on the level of concomitant immunosuppression which ought to be minimised if possible.

• Workup depends on the setting, HIV infection or transplantation and patient's symptoms.

• Local and systemic specific agents can be highly effective but are unable to cure the disease.

Abstract

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.

Introduction

These guidelines have been written under the auspices of the European Dermatology Forum (EDF), the European Association of Dermato-Oncology (EADO) and the European Organization for Research and Treatment of Cancer (EORTC) to help clinicians treating patients suffering from Kaposi's sarcoma (KS) in Europe, especially in countries where national guidelines are lacking.

It is our hope that these guidelines will assist health-care providers in defining local policies and standards of care and will foster progress towards an European consensus on the management of KS. It is not intended to replace national guidelines but to serve as basis for the development of these. The guidelines deal with all clinical settings of KS. The guidelines are also intended to promote the integration of care between medical and paramedical specialities for the benefit of patients.

These guidelines reflect the best published data available at the time the report was prepared. Caution should be exercised in interpreting the data; the results of future studies may require alteration of the conclusions or recommendations in this report. It may be necessary or even desirable to deviate from these guidelines in the interest of specific patients or under special circumstances. Just as adherence to the guidelines may not constitute defence against a claim of negligence, deviation from them should not necessarily be deemed negligent.