Metastatic Kaposi Sarcoma

  • Roberto N. Miranda
  • Joseph D. Khoury
  • L. Jeffrey Medeiros
Part of the Atlas of Anatomic Pathology book series (AAP)


Kaposi sarcoma (KS) is a distinctive type of vascular neoplasm that can involve any anatomic site and it is virtually always associated with human herpes virus-8 (HHV8) infection. HHV8, also known as Kaposi sarcoma-associated herpes virus, is uniformly expressed in KS lesions. The virus is distributed worldwide and the prevalence in the general population is variable, being highest in sub-Saharan Africa where more than 40 % of the population is infected. Seropositivity for HHV8 is less than 10 % in United States, northern Europe, and certain countries of Asia. The virus is transmitted via sexual or nonsexual routes. Infection with HHV8 is not sufficient to trigger KS, and other factors are involved in the pathogenesis including angiogenic factors, cytokines, and viral proteins expressed during latent and lytic phases of the viral life cycle. HHV8 likely infects progenitor cells of either blood vessel or lymphatic endothelium and usually establishes a latent infection. Kaposi sarcoma most often arises in the setting of immunodeficiency and particularly human immunodeficiency virus (HIV) infection. In patients with HIV, the transactivator of transcription (TAT) protein has mitogenic and modulating effects on KS cells and thus has a pathogenic role in these patients.


Human Immunodeficiency Virus Human Immunodeficiency Virus Infection Germinal Center Viral Life Cycle Lymphatic Endothelium 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    Mesri EA, Cesarman E, Boshoff C. Kaposi’s sarcoma and its associated herpesvirus. Nat Rev Cancer. 2010;10:707–19.PubMedCrossRefGoogle Scholar
  2. 2.
    Martin JN. The epidemiology of KSHV and its association with malignant disease. In: Arvin A, Campadelli-Fiume G, Mocarski E, et al., editors. Human herperviruses: biology, therapy and immunoprophylaxis. Cambridge: Cambridge University Press; 2007.Google Scholar
  3. 3.
    Zhang T, Shao X, Chen Y, et al. Human herpesvirus 8 seroprevalence, China. Emerg Infect Dis. 2012;18:150–2.PubMedCrossRefGoogle Scholar
  4. 4.
    Chatlynne LG, Ablashi DV. Seroepidemiology of Kaposi’s sarcoma-associated herpesvirus (KSHV). Semin Cancer Biol. 1999;9:175–85.PubMedCrossRefGoogle Scholar
  5. 5.
    Hengge UR, Ruzicka T, Tyring SK, et al. Update on Kaposi’s sarcoma and other HHV8 associated diseases. Part 2: pathogenesis, Castleman’s disease, and pleural effusion lymphoma. Lancet Infect Dis. 2002;2:344–52.PubMedCrossRefGoogle Scholar
  6. 6.
    Templeton AC. Kaposi’s sarcoma. Pathol Annu. 1981;16:315–36.PubMedGoogle Scholar
  7. 7.
    Sampaio MS, Cho YW, Qazi Y, et al. Posttransplant malignancies in solid organ adult recipients: an analysis of the U.S. National Transplant Database. Transplantation. 2012;94:990–8.PubMedCrossRefGoogle Scholar
  8. 8.
    Riva G, Luppi M, Barozzi P, et al. How I treat HHV8/KSHV-related diseases in posttransplant patients. Blood. 2012;120:4150–9.PubMedCrossRefGoogle Scholar
  9. 9.
    Ioachim HL, Adsay V, Giancotti FR, et al. Kaposi’s sarcoma of internal organs. A multiparameter study of 86 cases. Cancer. 1995;75:1376–85.PubMedCrossRefGoogle Scholar
  10. 10.
    Bueso-Ramos C. Metastatic Kaposi sarcoma. In: Medeiros LJ, editor. Diagnostic pathology: lymph nodes and spleen with Extranodal lymphomas. 1st ed. Altona/Manitoba: Amirsys, Inc; 2011. p. 12-24–9.Google Scholar
  11. 11.
    Naresh KN, Rice AJ, Bower M. Lymph nodes involved by multicentric Castleman disease among HIV-positive individuals are often involved by Kaposi sarcoma. Am J Surg Pathol. 2008;32:1006–12.PubMedCrossRefGoogle Scholar
  12. 12.
    Rosado FG, Itani DM, Coffin CM, Cates JM. Utility of immunohistochemical staining with FLI1, D2-40, CD31, and CD34 in the diagnosis of acquired immunodeficiency syndrome-related and non-acquired immunodeficiency syndrome-related Kaposi sarcoma. Arch Pathol Lab Med. 2012;136:301–4.PubMedCrossRefGoogle Scholar
  13. 13.
    Courville P, Simon F, Le Pessot F, et al. Detection of HHV8 latent nuclear antigen by immunohistochemistry. A new tool for differentiating Kaposi’s sarcoma from its mimics. Ann Pathol. 2002;22:267–76.PubMedGoogle Scholar
  14. 14.
    Mohanna S, Maco V, Bravo F, Gotuzzo E. Epidemiology and clinical characteristics of classic Kaposi’s sarcoma, seroprevalence, and variants of human herpesvirus 8 in South America: a critical review of an old disease. Int J Infect Dis. 2005;9:239–50.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Roberto N. Miranda
    • 1
  • Joseph D. Khoury
    • 1
  • L. Jeffrey Medeiros
    • 1
  1. 1.Department of HematopathologyThe University of Texas M.D. Anderson Cancer CenterHoustonUSA

Personalised recommendations