Synonyms
Angiosarcoma multiplex; Granuloma multiplex hemorrhagicum; Idiopathic multiple pigmented sarcoma of the skin
Definition
Kaposi sarcoma (KS), first described by Moritz Kaposi in 1872, is an angioproliferative disorder of endothelial origin, which can be considered as a low grade, locally aggressive tumor or as a tumor-like lesion. It is strictly associated to human herpesvirus 8 (HHV-8) infection (Micali et al. 2003).
Clinical Features
Incidence
Kaposi sarcoma is divided into four clinical subtypes: classical indolent, endemic African, iatrogenic, and AIDS-associated. The site of the lesion can vary from lower extremities in the classical form to lymph node involvement in the endemic type and to a more generalized localization in the two latter forms. In each subtype the involvement of external genitalia is very rare and when it occurs is usually related to HIV/AIDS-associated immunocompromised status. The cases arising in HIV-negative patients are exceedingly rare, with 19...
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References and Further Reading
Attwa, E., Gharib, K., Albalat, W., & Amer, A. (2016). Classical Kaposi sarcoma: Case reports with unusual presentation on the penis and scrotum. International Journal of Dermatology, 55(10), e533–e538.
Kaposi, M. (1872). Idiopathisches multiples pigmentsarkom der haut. Archiv für Dermatologie und Syphilis, 4, 265–273.
Mentzel, T., Knuutila, S., & Lamovec, J. (2013). Kaposi sarcoma. In C. D. M. Fletcher, J. A. C. Bridge, P. Hogendoorn, & F. Mertens (Eds.), WHO classification of tumours of soft tissue and bone (pp. 151–153). Lyon: IARC.
Micali, G., Nasca, M. R., De Pasquale, R., & Innocenzi, D. (2003). Primary classic Kaposi’s sarcoma of the penis: Report of a case and review. Journal of the European Academy of Dermatology and Venereology, 17(3), 320–323.
Weiss, S. W. (2001). Enzinger and Weiss’s soft tissue tumors. St. Louis: Mosby.
Yenice, M. G., Varnalı, E., Şeker, K. G., Kavak, A., & Tuğcu, V. (2018). Scrotal Kaposi’s sarcoma in HIV-negative patient: A case report and review of the literature. Turkish Journal of Urology, 44(2), 182–184.
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Taverna, C., Franchi, A. (2020). Kaposi Sarcoma. In: Raspollini, M.R., Lopez-Beltran, A. (eds) Uropathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-41894-6_5003
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DOI: https://doi.org/10.1007/978-3-030-41894-6_5003
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