Epidemiology of Kaposi sarcoma: review and description of the nonepidemic variant

Abstract

Kaposi sarcoma (KS) is a rare angioproliferative tumor whose etiology is associated with human herpesvirus 8 (HHV 8). KS lesions typically involve the skin or mucosal surfaces and are characterized by purplish, red-blue, or brown-black macules, papules, and nodules which are prone to bleeding and ulceration. Definitive diagnosis requires biopsy revealing characteristic angioproliferative features. There are four widely recognized types of KS, which are histologically indistinguishable but differ in epidemiology and prognosis. These include classic, endemic, iatrogenic, and epidemic. KS has been increasingly recognized in a new subgroup of patients: men who have sex with men (MSM) but who are HIV-seronegative human immuodeficiency virus-seronegative and have no identifiable immunodeficiency. This fifth variant of KS, termed nonepidemic KS, resembles classic KS in presentation and prognosis. In this literature review, we report the characteristics of nonepidemic KS based on all published cases and highlight the need for clinicians to recognize this new clinical variant.

Clinical features of Kaposi's sarcoma: experience from a Taiwanese medical center

“…Clinically, there are four recognized types of KS including classic, acquired immune deficiency syndrome (AIDS)‐associated (epidemic), African (endemic), and iatrogenic (immunosuppressive medication‐related) . KS lesions predominantly appear on the skin (lower extremities in particular) but can also involve noncutaneous sites in the body such as the oral cavity, gastrointestinal tract, and respiratory system …”

Approach to Peribronchovascular Disease on CT

“…Kaposi's sarcoma is categorized as classical, endemic or African, iatrogenic related to organ transplantation, and AIDS-associated or epidemic, or nonepidemic. 86 The human herpes virus 8 (HHV-8) causes Kaposi's sarcoma and is termed the Kaposi's sarcoma-associated herpesvirus. HHV-8 is also associated with multicentric Castleman's disease and pleural effusion lymphoma.…”

“…Visceral organ involvement, including the gastrointestinal tract and lungs, is particularly more aggressive in the endemic and AIDS-related forms, while the classic and nonepidemic form tends to be more indolent. 86 The classic form presents in Eastern European and South American older men, who have occasional visceral organ involvement and adenopathy. The endemic form occurs in younger patients, such as between 25 and 40 years and in children.…”

“…The endemic form occurs in younger patients, such as between 25 and 40 years and in children. 86 The nonepidemic form has been more frequently described in younger men and limited to the skin. 86 Pathological diagnosis is made by the presence of spindle cells forming vascular spaces around a vessel and proliferation of spindle cells.…”

Human leukocyte antigen polymorphisms and Kaposi's sarcoma‐associated herpesvirus infection outcomes: A call for deeper exploration

“…It is prevalent in sub‐Saharan Africa as well as in KSHV‐infected populations in Western countries. Finally, iatrogenic KS is essentially observed in patients undergoing immunosuppressive therapy and presents either chronically or with rapid progression (reviewed in the papers by Gallo, Dourmishev et al, and Vangipuram et al).…”