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Review
. 2015 Apr;42(2):223-46.
doi: 10.1053/j.seminoncol.2014.12.027. Epub 2014 Dec 31.

Kaposi sarcoma-associated herpesvirus-associated malignancies: epidemiology, pathogenesis, and advances in treatment

Manisha Bhutani  1 , Mark N Polizzotto  1 , Thomas S Uldrick  1 , Robert Yarchoan  2
Affiliations
Review

Kaposi sarcoma-associated herpesvirus-associated malignancies: epidemiology, pathogenesis, and advances in treatment

Manisha Bhutani et al. Semin Oncol. 2015 Apr.

Abstract

Kaposi sarcoma associated herpesvirus (KSHV), a γ2-herpesvirus, also known as human herpesvirus-8, is the etiologic agent of three virally associated tumors: Kaposi sarcoma, a plasmablastic form of multicentric Castleman disease (KSHV-MCD), and primary effusion lymphoma. These malignancies are predominantly seen in people with acquired immunodeficiencies, including acquired immunodeficiency syndrome and iatrogenic immunosuppression in the setting of organ transplantation, but can also develop in the elderly. Kaposi sarcoma (KS) is most frequent in regions with high KSHV seroprevalence, such as sub-Saharan Africa and some Mediterranean countries. In the era of combination antiviral therapy, inflammatory manifestations associated with KSHV-infection, including KSHV-MCD, a recently described KSHV-associated inflammatory cytokine syndrome and KS immune reconstitution syndrome also are increasingly appreciated. Our understanding of viral and immune mechanisms of oncogenesis continues to expand and lead to improved molecular diagnostics, as well as novel therapeutic strategies that employ immune modulatory agents, manipulations of the tumor microenvironment, virus-activated cytotoxic therapy, or agents that target interactions between specific virus-host cell signaling pathways. This review focuses on the epidemiology and advances in molecular and clinical research that reflects the current understanding of viral oncogenesis, clinical manifestations, and therapeutics for KSHV-associated tumors.

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Conflict of interest statement

Conflict of Interest: The spouse of one of the authors (R.Y.) is a co-inventor on an assay to measure KSHV v-IL6. This invention was made when this scientist was an employee of the US Government under 45 Code of Federal Regulations Part 7. All rights, title, and interest to this patent have been assigned to the U.S. Department of Health and Human Services. The government conveys a portion of the royalties it receives to its employee-inventors under the Federal Technology Transfer Act of 1986 (P.L. 99-502).

Figures

Figure 1
Figure 1
Kaposi sarcoma A) Multiple confluent violaceous papules on both lower extremities with tumor associated edema B) Nodular lesions on the hard palate C) Multiple oval, violaceous plaques on the upper extremities and trunk D) An ulcerated hyperkeratotic plaque with ulceration on the sole of the foot E) Esophagogastroduodenoscopy showing discrete, raised, violaceous plaques in the body of stomach F) Axial computerized tomography of chest shows diffuse peribronchovascular thickening, multiple nodules, and pleural effusion bilaterally; evaluation of the pleural fluid demonstrated an exudative, non-chylous effusion with no evidence of primary effusion lymphoma
Figure 2
Figure 2
Computerized tomography (CT) in KSHV-associated multicentric Castleman disease A) Coronal reformatted image from a contrast-enhanced CT scan of abdomen shows massive hepatosplenomegaly (upper limit of normal for spleen length = 12 cm) B) Coronal reformatted image from CT of the neck shows multiple enlarged bilateral cervical and supraclavicular lymph nodes
See this image and copyright information in PMC

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