Kaposi's sarcoma – still an enigma

Abstract

Kaposi's sarcoma (KS) is an unusual neoplasm that has proved to be an enigma in many ways since its original description in 1872. KS, a vascular tumour that is otherwise rare, is at present the most common neoplasm in patients with AIDS. The lesions contain spindle cells that share features with endothelial cells and smooth muscle cells and are in all likelihood primitive mesenchymal cells that can form vascular channels. These cells are monoclonal in origin indicating therefore that KS is a neoplasm. The presence of a novel type of human herpes virus, KS herpesvirus (KSHV) also called human herpesvirus type 8 (HHV8) in KS lesions support a viral ethiology. KS may be mistaken in the skin for an inflammatory or other lesion, thus skin biopsy is important for correct diagnosis, with the use of immunohistochemistry or molecular biology if needed. Radiation or interferon alpha dominate in the therapeutic approaches.

Latency-associated nuclear antigen expression and human herpesvirus-8 polymerase chain reaction in the evaluation of Kaposi sarcoma and other vascular tumors in HIV-positive patients

“…Formerly a rare tumor, the incidence of Kaposi sarcoma exploded with the onset of the AIDS epidemic, and Kaposi sarcoma is now the most common tumor seen in AIDS patients, with a disproportionate incidence among homosexual and bisexual men. [10][11][12] For this reason, an infectious etiology has long been suspected, and epidemiological, serologic, and molecular genetic studies over the past 10-15 years have identified a novel LANA expression in vascular tumors in HIV þ patients L Hammock et al herpesvirus, HHV-8, as the likely causative agent of Kaposi sarcoma. [10][11][12][13][14] HHV-8 is known to latently infect endothelial cells, as well as peripheral blood monocytes and Blymphocytes in patients with Kaposi sarcoma.…”

Classic Kaposi's sarcoma in southern Sardinia, Italy

“…The association with HHV8 infection in all forms of the disease (Babal and Pect, 2003) is consistent with a common pathologic process, but certain clinical-epidemiological aspects differentiate the course, the prognosis, and treatment of each form. Classical KS corresponds to the original description (Kaposi, 1872), and predominantly affects elderly males, with a higher incidence in southern and eastern European countries, with a predominance among Jews and people of Mediterranean descent (Ziegler et al, 1988, Mackie, 1992Franceschi and Geddes, 1995).…”

Preliminary Evaluation of in vivo Reflectance Confocal Microscopy Features of Kaposi’s Sarcoma

“…First described by Moritz Kaposi [1] in 1872, Kaposi’s sarcoma (KS) is an unusual vascular neoplasm with genetic, infectious, environmental and immunological pathogenesis [2,3]. KS presents polymorphic clinical features and evolution; it generally has a relatively benign, indolent course as in the genetic-linked, classic (Mediterranean) form.…”

“…Lesions may be limited to skin or sometimes disseminate to the mucous membranes, lymph nodes or viscera [4]. Typically, the KS lesion appears as brownish-red to purple or blue-colored patches, plaques or nodules in the distal lower extremities [2]. Moreover, there are clinical morphological variants, as in AIDS patients, which commonly present as small, pink, multifocal lesions located on the upper trunk and head [5].…”

“…HHV-8 genes encode homologies of several human genes that can participate in cell proliferation, producing mediators that stimulate the growth of spindle cells [2]. …”