Kaposi Sarcoma–Associated Herpesvirus-Associated Malignancies: Epidemiology, Pathogenesis, and Advances in Treatment
Open accessAbstract
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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Two other HHV8-associated neoplasms, primary effusion lymphoma and Castleman disease, have also been shown to be responsive to treatment with thalidomide analogues.
“Interestingly, two other HHV8-associated neoplasms, primary effusion lymphoma111 and Castleman disease112, have also been shown to be responsive to treatment with thalidomide analogues.”
From Cancer therapies based on targeted protein degradation — lessons learned with lenalidomide (2021) · cites this paper as [112]
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Flagged first, then by citations
Research works printed but not linked: 1
- [167]Welles L Saville MW Lietzau J Pluda JM Wyvill KM Feuerstein I Phase II trial with dose titration of paclitaxel for the therapy of human immunodeficiency virus-associated Kaposi’s sarcoma J Clin Oncol 1998 16 1112 21 9508198
Cited by
Sorted by citations
Polizzotto, M. N., Uldrick, T. S., Wyvill, K. M., Aleman, K., Peer, C. J., Bevans, M., Sereti, I., Maldarelli, F., Whitby, D., Marshall, V., et al. (2016) · Journal of Clinical Oncology · doi:10.1200/jco.2016.69.3812 · Cited by 138 · see citing works
Self-citationCancer therapies based on targeted protein degradation — lessons learned with lenalidomide
Jan, M., Sperling, A. S., & Ebert, B. L. (2021) · Nature Reviews Clinical Oncology · doi:10.1038/s41571-021-00479-z · Cited by 137 · see citing works
- Authors
- Bhutani, M., Polizzotto, M. N., Uldrick, T. S., & Yarchoan, R.
- Affiliations
- National Cancer Institute (US)
- Corresponding author
- Yarchoan, Robert
- Venue
- Seminars in Oncology
- Publisher
- Elsevier BV
- Published
- 2015-04
- Volume
- 42
- Issue
- 2
- Pages
- 223-246
- ISSN
- 0093-7754 (ISSN-L)
- PMC
- PMC6309362
- OpenAlex
- W2154435586
- Cited by
- 155
- References
- 209
- Citations by year
- 2026: 6 · 2025: 11 · 2024: 8 · 2023: 13 · 2022: 9 · 2021: 12 · 2020: 13 · 2019: 13 · 2018: 10 · 2017: 27 · 2016: 25 · 2015: 8
- FWCI
- 2.51
- Type
- journal article
- Language
- en
- Open access
- green
- europepmc.org
- Locations
- Seminars in Oncology (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Zenodo (CERN European Organization for Nuclear Research) (submittedVersion, public-domain)
- Funders
- National Institutes of Health
- National Cancer Institute (ZIABC010885, ZIABC010888)
- Topics
- Health Sciences › Medicine › Oncology › Viral-associated cancers and disorders
- Health Sciences › Medicine › Epidemiology › Herpesvirus Infections and Treatments
- Health Sciences › Medicine › Epidemiology › Cytomegalovirus and herpesvirus research
- Keywords
- Kaposi's sarcoma-associated herpesvirus, human herpesvirus 8, Kaposi's sarcoma, multicentric Castleman disease, primary effusion lymphoma, viral oncogenesis, immunodeficiency, HIV/AIDS, organ transplantation, antiviral therapy, tumor microenvironment, immunomodulatory therapy, molecular diagnosis
- MeSH terms
- Herpesviridae Infections, Humans, Neoplasms, Seroepidemiologic Studies, Herpesvirus 8, Human
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
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