Classic Kaposi sarcoma: Epidemiology, risk factors, pathology, and molecular pathogenesis
- Susan E Krown, MD
Susan E Krown, MD
- Member Emerita
- Memorial Sloan-Kettering Cancer Center
- Vice-Chair for International Activities
- The AIDS Malignancy Consortium
- Jasmeet Chadha Singh, MD
Jasmeet Chadha Singh, MD
- Attending Physician
- Memorial Sloan Kettering Cancer Center
- Commack, NY
- Section Editors
- Robert Maki, MD, PhD
Robert Maki, MD, PhD
- Section Editor — Bone and Soft Tissue Tumors
- Professor of Medicine
- Monter Cancer Center
- Hofstra-Northwell School of Medicine
- Thomas F DeLaney, MD
Thomas F DeLaney, MD
- Section Editor — Bone and Soft Tissue Tumors
- Professor of Radiation Oncology
- Harvard Medical School
- June K Robinson, MD
June K Robinson, MD
- Section Editor — Nonmelanoma Skin Cancer
- Professor of Clinical Dermatology
- Northwestern University Feinberg School of Medicine
Kaposi sarcoma (KS) is an angioproliferative disorder that requires infection with human herpes virus 8 (HHV-8), also known as Kaposi sarcoma-associated Herpes virus (KSHV), for its development [1-3]. The disease is named for Moritz Kaposi, a Hungarian dermatologist on the faculty of the University of Vienna, who first described the entity in 1872 as "idiopathic multiple pigmented sarcoma of the skin" .
KS is classified into four types based upon the clinical circumstances in which it develops: classic (the type originally described by Kaposi, which typically presents in middle or old age), endemic (several forms described in Sub-Saharan indigenous Africans prior to the acquired immunodeficiency syndrome [AIDS] epidemic), iatrogenic (a type associated with immunosuppressive drug therapy, typically seen in renal allograft recipients), and AIDS-associated (epidemic KS). The different epidemiologic and clinical aspects of these four types are summarized in the table (table 1).
This topic review will focus on the epidemiology, risk factors, pathology, and molecular pathogenesis of classic KS. The clinical presentation, diagnosis, staging, and treatment of classic KS is discussed elsewhere, as is AIDS-related KS and iatrogenic KS developing in the setting of immunosuppressive therapy. (See "Classic Kaposi sarcoma: Clinical features, staging, diagnosis, and treatment" and "AIDS-related Kaposi sarcoma: Clinical manifestations and diagnosis" and "AIDS-related Kaposi sarcoma: Staging and treatment" and "Development of malignancy following solid organ transplantation", section on 'Kaposi sarcoma'.)
Classic Kaposi sarcoma (CKS) affects men more often than women; the reported male to female ratio is approximately 3:1 [5-7]. Although cases have been described around the world, including in South America  and Asia , the disease is most often diagnosed in individuals from the Mediterranean basin and Central and Eastern Europe [10-12], or their descendants [5,13-15]. The highest incidence rates in Europe are in two Mediterranean Italian islands, Sardinia and Sicily .
An analysis of 2667 incident KS cases diagnosed between 1995 and 2002 and registered by 75 population-based European cancer registries that contributed to the RARECARE project ("Surveillance of rare cancer in Europe") estimated that there were 1642 new cases per year in the 27 countries comprising the European Union at that time and approximately 10,500 persons alive in Europe with a past diagnosis of KS as of the beginning of 2008 . The precise proportion of CKS was unknown; however, 852 diagnoses were made among individuals 65 years or older (most likely to represent CKS), 1147 among individuals less than 45 years of age (more likely to be human immunodeficiency virus [HIV]-associated), and the remaining 572 among individuals 45 to 64 years old .
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- RISK FACTORS
- Anti-HHV-8 antibodies and viremia
- Inherited variations in immunomodulating genes
- Immune activation
- Association with other malignancies
- Environmental factors
- MOLECULAR PATHOGENESIS
- INFORMATION FOR PATIENTS