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Kaposi’s Sarcoma of the Penis and Scrotum

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Rare Genitourinary Tumors

Abstract

Kaposi’s sarcoma (KS) is a mesenchymal tumor of blood and lymph vessels associated with infection of human herpesvirus type 8 (HHV-8). Four distinct clinical variants are found including (1) classic, (2) African or endemic, (3) AIDS related or epidemic, and (4) iatrogenic. Commonly affected sites include mucocutaneous surfaces, the oropharynx, genitals, lower extremities, and, in advanced stages, viscera.

For the period 1971–2014, we identified 44 reported cases of penile KS: 6 in HIV-seropositive patients, 29 in HIV-seronegative patients, and 9 patients with unspecified HIV status. Excisional biopsy and histology including intralesional HHV-8 testing were performed in all cases to establish the diagnosis. HIV-seropositive patients had more severe presentations including multiple lesions, ulceration, lesions extending to the groin, and non-discolored nodules. Treatment for these patients included maximizing highly active antiretroviral therapy (HAART). Patients with other clinical variants (HIV negative or unknown) had local treatment performed in 55.3 % (21 out of 38) of cases. Average follow-up was 14.2 months with local recurrence in two cases (2 out of 38, 5.3 %) and distant recurrence in five cases (5 out of 38, 13.2 %). In addition, six case reports of genitourinary KS in HIV-seropositive patients were reported. These cases had more severe presentations including nonpigmented nodules, multiple ulcerated lesions, lesions extending to groin area, deformity of the penis, and urethral stricture. More invasive treatment was attempted in addition to maximizing HAART.

Penile KS is a virus-induced neoplasm affecting primarily men who are HIV positive, have other forms of immunosuppression, or are of Mediterranean or African origin. These subgroups may have different patterns of presentation and require different treatment strategies.

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References

  1. Chang Y, et al. Identification of herpesvirus-like DNA sequences in AIDS-associated Kaposi’s sarcoma. Science. 1994;266:1865–9.

    Article  CAS  PubMed  Google Scholar 

  2. Russo J, et al. Nucleotide sequence of the Kaposi sarcoma-associated herpesvirus (HHV8). Proc Natl Acad Sci. 1996;93:14862–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Zhong W, et al. Restricted expression of Kaposi sarcoma-associated herpesvirus (human herpesvirus 8) genes in Kaposi sarcoma. Proc Natl Acad Sci. 1996;93:6641–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Nicholas J, et al. Kaposi’s sarcoma associated human herpesvirus-8 encodes homologues of macrophage inflammatory protein-1 and interleukin-6. Nat Med. 1997;3(3):287–92.

    Article  CAS  PubMed  Google Scholar 

  5. Neipel F, Albrecht J-C, Fleckenstein B. Cell-homologous genes in the Kaposi’s sarcoma-associated rhadinovirus human herpesvirus 8: determinants of its pathogenicity? J Virol. 1997;71(6):4187–92.

    CAS  PubMed  PubMed Central  Google Scholar 

  6. Renne R, et al. Lytic growth of Kaposi’s sarcoma-associated herpesvirus (human herpesvirus 8) in culture. Nat Med. 1996;2(3):342–6.

    Article  CAS  PubMed  Google Scholar 

  7. Schultz T. Epidemiology of Kaposi’s sarcoma-associated herpesvirus/human herpesvirus 8. Adv Cancer Res. 1999;76:121–60.

    Article  Google Scholar 

  8. Cohen A, et al. Kaposi’s sarcoma-associated herpesvirus: clinical, diagnostic, and epidemiological aspects. Crit Rev Clin Lab Sci. 2005;42(2):101–53.

    Article  PubMed  Google Scholar 

  9. Mainati M, et al. Human herpesvirus 8 (HHV-8/KSHV) and hematologic malignancies. Rev Clin Exp Hematol. 2003;7(4):375–405.

    Google Scholar 

  10. Antman K, Chang Y. Kaposi’s sarcoma. N Engl J Med. 2000;342:1027–38.

    Article  CAS  PubMed  Google Scholar 

  11. Ellis H. Moritz (Maurice) Kaposi: Kaposi’s sarcoma. J Perioper Pract. 2013;23(9):208.

    PubMed  Google Scholar 

  12. Oriel J. Mortiz Kaposi (1837–1902). Int J STD AIDS. 1997;8(11):715–7.

    Article  CAS  PubMed  Google Scholar 

  13. Chang Y, Moore P. Twenty years of KSHV. Viruses. 2014;6(11):4258–64.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Holubar K, Fatovic-Ferencic S. 1902–2002: a hundred years later. Moritz Kaposi 1837–1902: a historical reappraisal. Wien Klin Wochenschr. 2001;113:885–93.

    CAS  PubMed  Google Scholar 

  15. Sanders C. Kaposi’s sarcoma in retrospect. Genitourin Med. 1997;73(6):571–4.

    CAS  PubMed  PubMed Central  Google Scholar 

  16. Friedman-Kien A, et al. Disseminated Kaposi’s sarcoma syndrome in young homosexual men. J Am Acad Dermatol. 1981;5:468–71.

    Article  CAS  PubMed  Google Scholar 

  17. Moore P, Chang Y. Detection of herpesvirus-like DNA sequences in Kaposi’s sarcoma in patients with and without HIV infection. N Engl J Med. 1995;332:1181–5.

    Article  CAS  PubMed  Google Scholar 

  18. Boshoff C, Weiss R. In: Boshoff C, Weiss R, editors. Kaposi sarcoma herpesvirus: new perspectives, vol. 312. Heidelberg: Springer Science; 2006. 42.

    Google Scholar 

  19. Grulich A, Veral V, Swerdlow A. Kaposi’s sarcoma in England and Wales before the AIDS epidemic. Br J Cancer. 1992;66(6):1135–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Dictor M, Attewell R. Epidemiology of Kaposi’s sarcoma in Sweden prior to the acquired immunodeficiency syndrome. Int J Cancer. 1988;42:346–51.

    Article  CAS  PubMed  Google Scholar 

  21. Geddes M, et al. Kaposi’s sarcoma in Italy before and after the AIDS epidemic. Br J Cancer. 1994;69(2):333–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Touloumi G, et al. The epidemiologic profile of Kaposi’s sarcoma in Greece prior to and during the AIDS era. Int J Cancer. 1997;70:538–41.

    Article  CAS  PubMed  Google Scholar 

  23. Hjalgrim H, et al. High incidence of classical Kaposi’s sarcoma in Iceland and the Faroe Islands. Br J Cancer. 1998;77(7):1190–3.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  24. Cottoni F, De Marco M, Montesu A. Classical Kaposi’s sarcoma in north-east Sardinia: an overview from 1977 to 1991. Br J Cancer. 1996;73(9):1132–3.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. DiGiovanna J, Bijan S. Retrospective study of 90 cases with particular emphasis on the familial occurrence, ethnic background and prevalence of other diseases. Am J Med. 1981;71:779–83.

    Article  CAS  PubMed  Google Scholar 

  26. Cook-Mozaffari P, et al. The geographical distribution of Kaposi’s sarcoma and of lymphomas in Africa before the AIDS epidemic. Br J Cancer. 1998;78:1521–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. Taylor J, et al. Kaposi’s sarcoma in Uganda: geographic and ethnic distribution. Br J Cancer. 1972;26(6):483–97.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Ziegler J. Endemic Kaposi’s sarcoma in Africa and local volcanic soils. Lancet. 1993;342(8883):1348–51.

    Article  CAS  PubMed  Google Scholar 

  29. Montagnino G, et al. Clinical features and course of Kaposi’s sarcoma in kidney transplant patients: report of 13 cases. Am J Nephrol. 1994;14:121–6.

    Article  CAS  PubMed  Google Scholar 

  30. Parola L, et al. Kaposi’s sarcoma in renal-transplant recipients: experience at the Catholic University in Rome, 1988–1996. Dermatology. 1997;194:229–33.

    Article  Google Scholar 

  31. Penn I. Kaposi’s sarcoma in transplant recipients. Transplantation. 1997;64(5):669–73.

    Article  CAS  PubMed  Google Scholar 

  32. Luppi M, et al. HHV-8 infection in the transplantation setting: a concern only for solid organ transplant patients? Leuk Lymphoma. 2002;43(3):517–22.

    Article  PubMed  Google Scholar 

  33. Luppi M, et al. Bone marrow failure associated with human herpesvirus 8 infection after transplantation. N Engl J Med. 2000;343(19):1378–85.

    Article  CAS  PubMed  Google Scholar 

  34. Sarid R, et al. Detection of human herpesvirus-8 DNA in kidney allografts prior to the development of Kaposi’s sarcoma. Clin Infect Dis. 2001;32(10):1502–5.

    Article  CAS  PubMed  Google Scholar 

  35. Centers for Disease Control (CDC) (1981) Kaposi’s sarcoma and pneumocystis pneumonia among homosexual men – New York City and California. MMWR Morb Mortal Wkly Rep 30(25):305–8

    Google Scholar 

  36. Ganem D. KSHV and the pathogenesis of Kaposi sarcoma: listening to human biology and medicine. J Clin Invest. 2010;120(4):939–49.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  37. Selik R, Starcher E, Curran J. Opportunistic diseases reported in AIDS patients: frequencies, associations, and trends. AIDS. 1987;1(3):175–82.

    CAS  PubMed  Google Scholar 

  38. Arruda E, et al. Consensus of the Brazilian society of infectious diseases and Brazilian society of clinical oncology on the management and treatment of Kaposi’s sarcoma. Braz J Infect Dis. 2014;18(3):315–26.

    Article  PubMed  Google Scholar 

  39. Portsmouth S, et al. A comparison of regimens based on non-nucleoside reverse transcriptase inhibitors or protease inhibitors in preventing Kaposi’s sarcoma. AIDS. 2003;17(11):F17–22.

    Article  CAS  PubMed  Google Scholar 

  40. Lodi S, et al. Kaposi sarcoma incidence and survival among HIV-infected homosexual men after HIV seroconversion. J NCI. 2010;102:784–92.

    CAS  Google Scholar 

  41. Sharma S, Soneja M. HIV and immune reconstitution inflammatory syndrome (IRIS). Indian J Med Res. 2011;134(6):866–77.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  42. Wood C, Harrington Jr W. AIDS and associated malignancies. Cell Res. 2005;15(11–12):947–52.

    Article  PubMed  Google Scholar 

  43. Cannon G. Out of the box. Public Health Nutr. 2003;6(5):427–9.

    Article  PubMed  Google Scholar 

  44. Moore PS, Chang Y. Molecular virology of Kaposi’s sarcoma-associated herpesvirus. Philos Trans R Soc Lond B Biol Sci. 2001;356(1408):499–516.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  45. Dollard S, et al. Substantial regional differences in human herpesvirus 8 seroprevalence in sub-Saharan Africa: insights on the origin of the “Kaposi’s sarcoma Belt”. Int J Cancer. 2010;127:2395–401.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  46. Butler L, et al. Kaposi sarcoma associated herpesvirus (KSHV) seroprevalence in population based samples of African children: evidence for at least 2 patterns of KSHV transmission. J Infect Dis. 2009;200:430–8.

    Article  PubMed  PubMed Central  Google Scholar 

  47. Pica F, Volpi A. Transmission of human herpesvirus 8: an update. Curr Opin Infect Dis. 2007;20(2):152–6.

    Article  PubMed  Google Scholar 

  48. Al-Otaibi LM, et al. Extensive oral shedding of human herpesvirus 8 in a renal allograft recipient. Oral Microbiol Immunol. 2009;24(2):109–15.

    Article  CAS  PubMed  Google Scholar 

  49. Bourboulia D, et al. Serologic evidence for mother-to-child transmission of Kaposi sarcoma-associated herpesvirus infection. JAMA. 1998;280(1):31–2.

    Article  CAS  PubMed  Google Scholar 

  50. Andreoni M, et al. High seroprevalence of antibodies to human herpesvirus-8 in Egyptian children: evidence of nonsexual transmission. J Natl Cancer Inst. 1999;91(5):465–9.

    Article  CAS  PubMed  Google Scholar 

  51. Regamey N, et al. Transmission of human herpesvirus 8 infection from renal-transplant donors to recipients. N Engl J Med. 1998;339(19):1358–63.

    Article  CAS  PubMed  Google Scholar 

  52. Ariza-Heredia EJ, Razonable RR. Human herpes virus 8 in solid organ transplantation. Transplantation. 2011;92(8):837–44.

    Article  PubMed  Google Scholar 

  53. Bobroski S, Bagasra A, Patel D, Saikumari P, Memoli M, Abbey MV, Wood C, Sosa C, Bagasra O. Localization of human herpesvirus type 8 (HHV-8) in the Kaposi’s sarcoma tissues and the semen of specimens of HIV-1 infected and uninfected individuals by utilizing in situ polymerase chain reaction. J Reprod Immunol. 1998;41:149–60.

    Article  CAS  PubMed  Google Scholar 

  54. Lin JC LS, Mar EC, Pellett PE, Stamney F, Stewart J, Spira TJ. Is Kaposi’s sarcoma-associated herpesvirus detectable in semen of HIV-infected homosexual men? Lancet. 1995;346:160–2.

    Google Scholar 

  55. Viviano E, Vitale F, Ajello F, Perna AM, Villafrate MR, Bonura F, Arico M, Mazzola G, Romano N. Human herpesvirus type 8 DNA sequences in biological samples of HIV-positive and negative individuals in Sicily. AIDS. 1997;11:607–12.

    Article  CAS  PubMed  Google Scholar 

  56. Diamond C, Brodie S, Krieger JN, Huang ML, Koelle DM, Diem K, Muthui D, Corey L. Human herpesvirus 8 in the prostate glands of men with Kaposi’s sarcoma. J Virol. 1998;72(7):6223–7.

    CAS  PubMed  PubMed Central  Google Scholar 

  57. Corbellino M, Galli M, Parravicini C. Absence of HHV-8 in prostate and semen. N Engl J Med. 1996;335:1237–9.

    Article  CAS  PubMed  Google Scholar 

  58. Monini P, de Lellis L, Fabris M, Rigolin F, Cassai E. Kaposi’s sarcoma-associated herpesvirus DNA sequences in prostate tissue and human semen. N Engl J Med. 1996;334(18):1168–72.

    Article  CAS  PubMed  Google Scholar 

  59. Lowe F, Lattimer D. Kaposi’s sarcoma of the penis in patients with acquired immunodeficiency syndrome. J Urol. 1989;142:1475–7.

    CAS  PubMed  Google Scholar 

  60. Micali G, et al. Primary classic Kaposi’s sarcoma of the penis: report of a case and review. J Eur Acad Dermatol Venereol. 2003;17:320–3.

    Article  CAS  PubMed  Google Scholar 

  61. Beral V, et al. Kaposi’s sarcoma among persons with AIDS: a sexually transmitted infection? Lancet. 1990;335(8682):123–8.

    Article  CAS  PubMed  Google Scholar 

  62. Grulich A, et al. Kaposi’s sarcoma-associated herpesvirus: a sexually transmissible infection? J AIDS Hum Retrovirol. 1999;20:387–93.

    CAS  Google Scholar 

  63. Beral V, et al. Risk of Kaposi’s sarcoma and sexual practices associated with faecal contact in homosexual or bisexual men with AIDS. Lancet. 1992;339:632–5.

    Article  CAS  PubMed  Google Scholar 

  64. Gupta P, et al. Detection of Kaposi’s sarcoma herpesvirus DNA in semen of homosexual men with Kaposi’s sarcoma. AIDS. 1996;10:1596–8.

    Article  CAS  PubMed  Google Scholar 

  65. Corey L, et al. HHV-8 infection: a model for reactivation and transmission. Rev Med Virol. 2002;12:47–63.

    Article  PubMed  Google Scholar 

  66. Pauk J, Huang M, Brodie SJ, Wald A, Koelle DM, Schacker T, Celum C, Selke S, Corey L. Mucosal shedding of human herpesvirus 8 in men. N Engl J Med. 2000;343(19):1369–77.

    Article  CAS  PubMed  Google Scholar 

  67. Dupin N, et al. Herpesvirus-like DNA sequences in patients with Mediterranean Kaposi’s sarcoma. Lancet. 1995;345(8952):1043–4.

    Article  Google Scholar 

  68. Boshoff C, et al. Kaposi’s sarcoma-associated herpesvirus in HIV negative Kaposi’s sarcoma. Lancet. 1995;345(8956):1043–4.

    Article  CAS  PubMed  Google Scholar 

  69. Collandre H, et al. Kaposi’s sarcoma and new herpesvirus. Lancet. 1995;345(8956):1043.

    Article  CAS  PubMed  Google Scholar 

  70. Kedes D, et al. The seroepidemiology of human herpesvirus 8 (Kaposi’s sarcoma-associated herpesvirus): distribution of infection in KS risk groups and evidence for sexual transmission. Nat Med. 1996;2(8):918–24.

    Article  CAS  PubMed  Google Scholar 

  71. Gao SJ, et al. KSHV antibodies among Americans, Italians and Ugandans with and without Kaposi’s sarcoma. Nat Med. 1996;2(8):925–8.

    Article  CAS  PubMed  Google Scholar 

  72. Simpson G, et al. Prevalence of Kaposi’s sarcoma associated herpesvirus infection measured by antibodies to recombinant capsid protein and latent immunofluorescence antigen. Lancet. 1996;348(9035):1133–8.

    Article  CAS  PubMed  Google Scholar 

  73. Moore P, et al. Kaposi’s sarcoma-associated heepwacieua indwxrion peioe ro onawr od Kaposi’s sarcoma. AIDS. 1996;10(2):175–80.

    Article  CAS  PubMed  Google Scholar 

  74. Martin J, et al. Sexual transmission and the natural history of human herpesvirus 8 infection. N Engl J Med. 1998;338(14):948–54.

    Article  CAS  PubMed  Google Scholar 

  75. Edelman D. Human herpesvirus 8 – a novel human pathogen. Virol J. 2005;2:78.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  76. Cesarman E, et al. Kaposi’s sarcoma-associated herpesvirus-like DNA sequences in AIDS-related body cavity-based lymphomas. N Engl J Med. 1995;332:1186–91.

    Article  CAS  PubMed  Google Scholar 

  77. Sturzl M, et al. Expression of HHV-8 latency-associated T0.7 RNA in spindle cells and endothelial cells of AIDS-associated, classical and African Kaposi’s sarcoma. Int J Cancer. 1997;72(1):68–71.

    Article  CAS  PubMed  Google Scholar 

  78. Coleman C, Nealy M, Tibbetts S. Immature and transitional B cells are latency reservoirs for a gammaherpesvirus. J Virol. 2010;84(24):13045–52.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  79. Ciufo D, et al. Spindle cell conversion by Kaposi’s sarcoma associated herpesvirus: formation of colonies and plaques with mixed lytic and latent gene expression in infected primary dermal microvascular endothelial cell cultures. J Virol. 2001;75(12):5614–26.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  80. Grossman C, et al. Activation of NF-kb by the latent v-FLIP gene of KSHV is required for the spindle shape of virus-infected endothelial cells and contributes to their pro-inflammatory phenotype. J Virol. 2006;80(14):7179–85.

    Article  CAS  Google Scholar 

  81. Wen K, Damania B. Kaposi sarcoma-associated herpesvirus (KSHV): molecular biology and oncogenesis. Cancer Lett. 2010;289(2):140–50.

    Article  CAS  PubMed  Google Scholar 

  82. Ballestas M, Chatis P, Kaye K. Efficient persistence of extrachromosomal KSHV DNA mediated by latency-associated nuclear antigen. Science. 1999;284(5414):641–4.

    Article  CAS  PubMed  Google Scholar 

  83. Ballestas M, Kaye K. Kaposi’s sarcoma-associated herpesvirus latency-associated nuclear antigen 1 mediates episome persistence through cis-acting terminal repeat (TR) sequence and specifically binds TR DNA. J Virol. 2001;75(7):3250–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  84. Cotter M, Robertson E. The latency-associated nuclear antigen tethers the Kaposi’s sarcoma-associated herpesvirus genome to host chromosomes in body cavity-based lymphoma cells. Virol J. 1999;264(2):254–64.

    Article  CAS  Google Scholar 

  85. Cotter M, Subramanian C, Robertson E. The Kaposi’s sarcoma-associated herpesvirus latency-associated nuclear antigen binds to specific sequences at the left end of the viral genome through its carboxy-terminus. Virol J. 2001;291(2):241–59.

    Article  CAS  Google Scholar 

  86. Garber A, et al. DNA binding and modulation of gene expression by the latency-associated nuclear antigen of Kaposi’s sarcoma-associated herpesvirus. J Virol. 2001;75(17):7882–92.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  87. Garber A, Hu J. Latency-associated nuclear antigen (LANA) cooperatively binds to two sites within the terminal repeat, and both sites contribute to the ability of LANA to suppress transcription and to facilitate DNA replication. J Biol Chem Renne R. 2002;277(30):27401–11.

    Article  CAS  Google Scholar 

  88. Friborg JJ, et al. p53 inhibition by the LANA protein of KSHV protects against cell death. Nature. 1999;402(6764):889–94.

    CAS  PubMed  Google Scholar 

  89. Radkov S, Kellam P, Boshoff C. The latent nuclear antigen of Kaposi sarcoma-associated herpesvirus targets the retinoblastoma-E2F pathway and with the oncogene Hras transforms primary rat cells. Nat Med. 2000;6(10):1121–7.

    Article  CAS  PubMed  Google Scholar 

  90. Chang Y, et al. Cyclin encoded by KS herpesvirus. Nature. 1996;82(6590):410.

    Article  Google Scholar 

  91. Swanton C, et al. Herpes viral cyclin/Cdk6 complexes evade inhibition by CDK inhibitor proteins. Nature. 1997;390:184–7.

    Article  CAS  PubMed  Google Scholar 

  92. Ellis M, et al. Degradation of p27(Kip) cdk inhibitor triggered by Kaposi’s sarcoma virus cyclin-cdk6 complex. EMBO J. 1999;18(3):644–53.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  93. Mann D, et al. Modulation of p27(Kip1) levels by the cyclin encoded by Kaposi’s sarcoma-associated herpesvirus. EMBO J. 1999;18(3):654–63.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  94. Thome M, et al. Viral FLICE-inhibitory proteins (FLIPs) prevent apoptosis induced by death receptors. Nature. 1997;386(6624):517–21.

    Article  CAS  PubMed  Google Scholar 

  95. Muralidhar S, et al. Identification of kaposin (open reading frame K12) as a human herpesvirus 8 (Kaposi’s sarcoma associated herpesvirus) transforming gene. J Virol. 1998;72(6):4980–8.

    CAS  PubMed  PubMed Central  Google Scholar 

  96. Monini P, et al. Reactivation and persistence of human herpesvirus-8 infection in B cells and monocytes by TH-1 cytokines. Blood. 1999;93(12):4044–58.

    CAS  PubMed  Google Scholar 

  97. Duus K, et al. Wild-type Kaposi’s sarcoma-associated herpesvirus isolated from the oropharynx of immune-competent individuals has tropism for cultured oral epithelial cells. J Virol. 2004;78(8):4074–84.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  98. Casper C, et al. Frequent and asymptomatic oropharyngeal shedding of human herpesvirus 8 among immunocompetent men. J Infect Dis. 2007;195(1):30–6.

    Article  PubMed  Google Scholar 

  99. XU J, et al. The Epstein-Barr virus (EBV) envelope glycoprotein gp350/220 specific antibody reactivities in the sera of patients with different EBV-associated diseases. Int J Cancer (Pred Oncol). 1998;79:481–6.

    Article  CAS  Google Scholar 

  100. Kumar P, et al. Higher levels of neutralizing antibodies against KSHV in KS patients compared to asymptomatic individuals from Zambia. PLoS One. 2013;8(8):e71254.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  101. Kimball L, et al. Reduced levels of neutralizing antibodies to Kaposi sarcoma-associated herpesvirus in persons with a history of Kaposi sarcoma. J Infect Dis. 2004;189:2016–22.

    Article  CAS  PubMed  Google Scholar 

  102. Ganem D. KSHV and Kaposi’s sarcoma: the end of the beginning? Cell. 1997;91:157–60.

    Article  CAS  PubMed  Google Scholar 

  103. Kaposi M. Idiopathisches multiples pigmentsarkom her haut. Arch Dermatol Shypillol. 1872;4:265–73.

    Article  Google Scholar 

  104. Regezi J, et al. Oral Kaposi’s sarcoma: a 10-year retrospective histopathologic study. J Oral Pathol Med. 1993;22(7):292–7.

    Article  CAS  PubMed  Google Scholar 

  105. Regezi J, et al. Human immunodeficiency virus-associated oral Kaposi’s sarcoma. A heterogeneous cell population dominated by spindle-shaped endothelial cells. Am J Pathol. 1993;143(1):240–9.

    CAS  PubMed  PubMed Central  Google Scholar 

  106. Herndier B, Ganem D. The biology of Kaposi’s sarcoma. Cancer Treat Res. 2001;104:89–126.

    Article  CAS  PubMed  Google Scholar 

  107. Grayson W, Pantanowitz L. Histological variants of cutaneous Kaposi sarcoma. Diagn Pathol. 2008;3:31.

    Article  PubMed  PubMed Central  Google Scholar 

  108. Hanahan D, Folkman J. Patterns and emerging mechanisms of the angiogenic switch during tumorigenesis. Cell. 1996;86(3):353–64.

    Article  CAS  PubMed  Google Scholar 

  109. Staskus K, et al. Kaposi’s sarcoma-associated herpesvirus gene expression in endothelial (spindle) tumor cells. J Virol. 1997;71(1):715–9.

    CAS  PubMed  PubMed Central  Google Scholar 

  110. Ensoli B, Sturzl M. Kaposi’s sarcoma: a result of the interplay among inflammatory cytokines, angiogenic factors and viral agents. Cytokine Growth Factor Rev. 1998;9(1):63–83.

    Article  CAS  PubMed  Google Scholar 

  111. Ensoli B, et al. Biology of Kaposi’s sarcoma. Eur J Cancer. 2001;37(10):1251–69.

    Article  CAS  PubMed  Google Scholar 

  112. Ensoli B, et al. AIDS-Kaposi’s sarcoma-derived cells express cytokines with autocrine and paracrine growth effects. Science. 1989;243(4888):223–6.

    Article  CAS  PubMed  Google Scholar 

  113. Brooks J. Kaposi’s sarcoma: a reversible hyperplasia. Lancet. 1986;2(8519):1309–11.

    Article  CAS  PubMed  Google Scholar 

  114. Duprez R, et al. Evidence for a multiclonal origin of multicentric advanced lesions of Kaposi sarcoma. J Natl Cancer Inst. 2007;99(14):1086–94.

    Article  CAS  PubMed  Google Scholar 

  115. Kirshner J, et al. The expression of the ORF 74 (G protein coupled receptor) gene of Kaposi’s sarcoma-associated herpesvirus: implications for KS pathogenesis. J Virol. 1999;73(7):6006–14.

    CAS  PubMed  PubMed Central  Google Scholar 

  116. Cesarman E, Mesri E, Gershengorn M. Viral G protein-coupled receptor and Kaposi’s sarcoma: a model of paracrine neoplasia? J Exp Med. 2000;191(3):417–22.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  117. Krown S. Therapy of AIDS-associated Kaposi’s sarcoma: targeting pathogenetic mechanisms. Hematol Oncol Clin N Am. 2003;17:763–83.

    Article  Google Scholar 

  118. Mandong B, et al. Histopathological study of Kaposi’s sarcoma in JOS: a 16-year review. Ann Afr Med. 2004;3(4):174–6.

    Google Scholar 

  119. Wabinga H, et al. Trends in cancer incidence in Kyadondo County, Uganda, 1960–1997. Br J Cancer. 2000;82(9):1585–92.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  120. Bassett M, et al. Cancer in the African population of Harare, Zimbabwe, 1990–1992. Int J Cancer. 1995;63(1):29–36.

    Article  CAS  PubMed  Google Scholar 

  121. Ahmed A, Muktar H, Bugaje M. Epidemiological and clinical features of AIDS-Associated Kaposi’s Sarcoma in Northern Nigeria. Arch Int Surg. 2013;3(1):29–34.

    Article  Google Scholar 

  122. Chalya P, et al. Kaposi’s sarcoma: a 10-year experience with 248 patients at a single tertiary care hospital in Tanzania. BMC Res Notes. 2015;8:1–10.

    Article  Google Scholar 

  123. Santos-Fortuna E, Caterino-de-Araujo A. Confirming shedding of human herpesvirus 8 in urine from infected patients in Brazil. J Clin Microbiol. 2005;43(2):1008.

    Article  PubMed  PubMed Central  Google Scholar 

  124. Biermann C, Gasser T, Rutishauser G. Kaposi’s sarcoma of the urinary bladder after kidney transplantation. Helv Chir Acta. 1992;59(3):503–5.

    CAS  PubMed  Google Scholar 

  125. Rha S, et al. Kaposi sarcoma involving a transplanted kidney, ureter and urinary bladder: ultrasound and CT findings. Br J Radiol. 2000;73:1221–3.

    Article  CAS  PubMed  Google Scholar 

  126. Yangm C, et al. Acute graft dysfunction due to Kaposi sarcoma involving the bladder in a renal transplant recipient. Nephrol Dial Transplant. 2001;16:625–7.

    Article  Google Scholar 

  127. Lebovitch S, Mydlo J. HIV-AIDS: urologic considerations. Urol Clin North Am. 2008;35:59–68.

    Article  PubMed  Google Scholar 

  128. John H, Pestalozzi D, Hauri D. Kaposi sarcoma of the glans penis with meatal obstruction. Case Report and literature review (article in German). Swiss Surg. 1996;3:134–6.

    PubMed  Google Scholar 

  129. Antunes A, et al. Deep-seated sarcoma of the penis. Int Braz J Urol. 2005;31(3):245–50.

    Article  PubMed  Google Scholar 

  130. Dorffel J. Arch Dermatol Syph. 1932;26:608.

    Article  Google Scholar 

  131. Hopkins J, Hudson P. Kaposi’s sarcoma: penile and scrotal lesions. Br J Urol. 1953;25(3):233–6.

    Article  CAS  PubMed  Google Scholar 

  132. Linker D, Lieberman P, Grabstald H. Kaposi’s sarcoma of the genitourinary tract. Urology. 1975;5:684–7.

    Article  CAS  PubMed  Google Scholar 

  133. Marquart KH, Oehlschlaegel G, Engst R. Disseminated Kaposi’s sarcoma that is not associated with acquired immunodeficiency syndrome in a bisexual man. Arch Pathol Lab Med. 1986;110:346–7.

    CAS  PubMed  Google Scholar 

  134. Zambolin T, Simeone C, Baronchelli C, Cunico SC. Kaposi’s sarcoma of the penis. Br J Urol. 1989;63:645–6.

    Article  CAS  PubMed  Google Scholar 

  135. Lands RH, Ange D, Hartman DL. Radiation therapy for classic Kaposi’s sarcoma presenting only on the glans penis. J Urol. 1992;147:468–70.

    CAS  PubMed  Google Scholar 

  136. Myslovaty B, Kyzer S, Koren R, Chaimoff C. Kaposi sarcoma limited to the glans penis. Plast Reconstr Surg. 1993;92:764.

    Article  CAS  PubMed  Google Scholar 

  137. Guy M, Singer D, Barzilai N, Eisenkraft S. Primary classic Kaposi’s sarcoma of glans penis-appearance on magnetic resonance imaging. Br J Urol. 1994;74:521–2.

    Article  CAS  PubMed  Google Scholar 

  138. Grunwald MH, Amichai B, Halevy S. Purplish penile papule as a presenting sign of Kaposi’s sarcoma. Br J Urol. 1994;74:517.

    Article  CAS  PubMed  Google Scholar 

  139. Ruszczack Z, Stadler R, Schwartz RA. Kaposi’s sarcoma limited to the penis treated with cobalt-60 radiotherapy. J Med. 1996;27:211–20.

    Google Scholar 

  140. Koyuncuoglu M, Yalcin N, Ozkan S, Kirkali Z. Primary Kaposi’s sarcoma of the glans penis. Br J Urol. 1996;77:614–5.

    Article  CAS  PubMed  Google Scholar 

  141. Chun Y, Chang SN, Park WH. A case of classical Kaposi’s sarcoma of the penis showing a good response to high energy pulsed carbon dioxide laser therapy. J Dermatol. 1999;26:240–3.

    Article  CAS  PubMed  Google Scholar 

  142. Schwartz RA, Cohen J, Watson RA, et al. Penile Kaposi’s sarcoma preceded by chronic penile lymphoedema. Br J Dermatol. 2000;142:153–6.

    Article  CAS  PubMed  Google Scholar 

  143. Chitale SV, Peat D, Meaden JD, Johnson HB, Burgess NA. Kaposi’s sarcoma of the glans penis in an HIV negative patient. Int Urol Nephrol. 2002;34:251–3.

    Article  CAS  PubMed  Google Scholar 

  144. Pacifico A, Piccolo D, Fargnoli MC, Peris K. Kaposi’s sarcoma of the glans penis in an immunocompetent patient. Eur J Dermatol. 2003;13:582–3.

    PubMed  Google Scholar 

  145. Morelli L, Pusiol T, Piscioli F, Höfler H, Weirich G, Werner M, Cabras AD. Herpesvirus 8-associated penile Kaposi’s sarcoma in an HIV-negative patient: first report of a solitary lesion. Am J Dermatopathol. 2003;25(1):28–31.

    Article  PubMed  Google Scholar 

  146. Serrano C, Sánchez G, del Mar Serrano M, Linares J, Dulanto C, Naranjo R. Violaceous nodules and plaques on the scrotum and thigh. Actas Dermosifiliogr. 2005;96(2):127–9. [Spanish].

    Article  PubMed  Google Scholar 

  147. Ekmekci TR, Kendirci M, Kizilkay O, Koslu A. Sildenafil citrate-aided radiotherapy for the treatment of Kaposi’s sarcoma of the penis. J Eur Acad Dermatol Venereol. 2005;19(5):603–4.

    Article  CAS  PubMed  Google Scholar 

  148. Gonen M, Cenker A, Kiyici H, Kalkan M. Penile Kaposi’s sarcomas in a circumcised and HIV-seronegative patient. Int J Urol. 2006;13:318–20.

    Article  PubMed  Google Scholar 

  149. Zargari O. Exclusive penile Kaposi’s sarcoma: report of an HIV-negative man successfully treated with radiotherapy. J Eur Acad Dermatol Venereol. 2006;20(3):318–20.

    Article  CAS  PubMed  Google Scholar 

  150. Curatolo P, Mancini M, Ruggiero A, Clerico R, Di Marco P, Calvieri S. Successful treatment of penile Kaposi’s sarcoma with electrochemotherapy. Dermatol Surg. 2008;34:839–43.

    CAS  PubMed  Google Scholar 

  151. Mukai MM, Chaves T, Caldas L, Fillus Neto J, Santamaria JR. Sarcoma de Kaposi primário do pênis. An Bras Dermatol. 2009;84(5):524–6.

    Article  PubMed  Google Scholar 

  152. Kim KH, Choi J, Ryu KH, Kang IH, Leng YH, Lee JW, Lee JW, Kim YJ, Lee JK. Primary classic Kaposi’s sarcoma of the penis in an HIV-negative patient. Korean J Urol. 2010;51(11):803–6.

    Article  PubMed  PubMed Central  Google Scholar 

  153. Seleit I, Attia A, Maraee A, Samaka R, Bakry O, Eid E. Isolated Kaposi sarcoma in two HIV negative patients. J Dermatol Case Rep. 2011;5(2):24–6.

    Article  PubMed  PubMed Central  Google Scholar 

  154. Cecchi R, Troiano M, Ghilardi M, Bartoli L. Kaposi sarcoma of the penis in an HIV-negative patient. J Cutan Med Surg. 2011;15(2):118–20.

    Article  PubMed  Google Scholar 

  155. Emadi SN, Babamahmoodi F, Poursaleh Z, Sayad-Noori SS, Soroush MR, Maleki AR, Izadi M, Khodaei-Ardakan MR, Emadi SE. Sézary syndrome, Kaposi sarcoma and generalized dermatophytosis 15 years after sulfur mustard gas exposure. J Dermatol Case Rep. 2012;6(3):86–9.

    Article  PubMed  PubMed Central  Google Scholar 

  156. Kamari H, Bagher-Tabrizi, Yaghoobi M. Primary Kaposi sarcoma of penis in HIV negative patient. Iran J Cancer Prev. 2012;5(4):225–7.

    Google Scholar 

  157. Kampantais S, Gourvas V, Ioannidis S. Penile Kaposi’s sarcoma in a HIV negative HHV-8 positive man. Hippokratia. 2013;17(1):96.

    CAS  PubMed  PubMed Central  Google Scholar 

  158. Ozmen H, Baba D, Kacagan C, Kayikci A, Cam K. Case report: HIV negative isolated scrotal Kaposi’s sarcoma. Int J Surg Case Rep. 2014;5(12):1086–7.

    Article  PubMed  PubMed Central  Google Scholar 

  159. Girgis AS, Bergman H, Rosenthal H, Solomon L. Unusual penile malignancies in circumcised Jewish men. J Urol. 1973;110:696–702.

    CAS  PubMed  Google Scholar 

  160. Vyas S, Manabe T, Herman JR, Newman HR. Kaposi’s sarcoma of the scrotum. Urology. 1976;8(1):82–5.

    Article  CAS  PubMed  Google Scholar 

  161. Conger K, Sporer A. Kaposi sarcoma limited to glans penis. Urology. 1985;26:173–5.

    Article  CAS  PubMed  Google Scholar 

  162. Maiche AG, Holsti P, Grohn P, Wasenius P. Kaposi’s sarcoma of penis. Br J Urol. 1986;58:557.

    Article  CAS  PubMed  Google Scholar 

  163. Jaimowich L, Calb I, Kaminsky A. Kaposi’s sarcoma of the conjunctiva. J Am Acad Dermatol. 1986;14:589–92.

    Article  Google Scholar 

  164. Casado M, Jimenez F, Borbujo J, Almagro M. Spontaneous healing of Kaposi’s angiosarcoma of the penis. J Urol. 1988;139:1313–4.

    CAS  PubMed  Google Scholar 

  165. Berkmen F, Celebioğlu A. Adult genitourinary sarcomas: a report of seventeen cases and review of the literature. J Exp Clin Cancer Res. 1997;16:45–8.

    CAS  PubMed  Google Scholar 

  166. Kagu MB, Nggada HA, Garandawa HI, Askira BH, Durosinmi MA. AIDS-associated Kaposi’s sarcoma in Northeastern Nigeria. Singapore Med J. 2006;47(12):1069–74.

    CAS  PubMed  Google Scholar 

  167. Zargar-Shoshtari K, et al. Establishing criteria for bilateral pelvic lymph node dissection in the management of penile cancer: lessons learned from an International Multicenter Collaboration. J Urol. 2015;194:696–701.

    Article  PubMed  Google Scholar 

  168. Pinto-Almeida T, Torres T, Rosmaninho A, Sanches M, Alves R, Caetano M, Selores M. Letter: penile Kaposi sarcoma: a case of complete resolution with highly active antiretroviral therapy alone. Dermatol Online J. 2011;17(6):12.

    PubMed  Google Scholar 

  169. Asuquo ME, Ogunkeyede A, Bassey EE, Ebughe G. Kaposi sarcoma: changing trend in Calabar, southeastern Nigeria. Ann Afr Med. 2008;7(3):98–101.

    Article  CAS  PubMed  Google Scholar 

  170. Ruocco E, Valenzano F, Brunetti G, Schwartz RA, Ruocco V. Monolesional Kaposi sarcoma at the site of slow healing herpes zoster in an HIV+ patient: immunocompromised district in an immunocompromised patient. Am J Dermatopathol. 2013;35(8):e139–24.

    Article  PubMed  Google Scholar 

  171. Swierzewski III SJ, Wan J, Boffini A, Faerber GJ. The management of meatal obstruction due to Kaposi’s sarcoma of the glans penis. J Urol. 1993;150(1):193–5.

    PubMed  Google Scholar 

  172. Klein LT, Lowe F. Penile gangrene associated with extensive Kaposi’s sarcoma in patients with acquired immunodeficiency syndrome. Urology. 1995;46(3):425–8.

    Article  CAS  PubMed  Google Scholar 

  173. Hayes CW, Clark RM, Politano VA. Kaposi’s sarcoma of the penis. J Urol. 1971;105:525–7.

    CAS  PubMed  Google Scholar 

  174. Lebari D, Gohil J, Patnaik L, Wasef W. Isolated penile Kaposi’s sarcoma in an HIV-positive patient stable on treatment for three years. Int J STD AIDS. 2014;25(8):607–10.

    Article  PubMed  Google Scholar 

  175. Woldrich J, et al. Penile Kaposi sarcoma in the state of California. Can Urol Assoc J. 2012;19(2):6178–82.

    Google Scholar 

  176. Ritchie A, Foster P, Fowler S. Penile cancer in the UK: clinical presentation and outcomes in 1998/99. BJU Int. 2004;94(9):1248–52.

    Article  PubMed  Google Scholar 

  177. Heyns C, Mendoza-Valdes A, Pompeo A. Diagnosis and staging of penile cancer. Urology. 2010;76(2 Suppl 1):S15–23.

    Article  PubMed  Google Scholar 

  178. Kavak A, Akman R, Alper M, Buyukbabani N. Penile Kaposi’s sarcoma in a human immunodeficiency virus-seronegative patient. Br J Dermatol. 2001;144(1):207–8.

    Article  CAS  PubMed  Google Scholar 

  179. Restrepo CS, Ocazionez D. Kaposi’s sarcoma: imaging overview. Semin Ultrasound CT MR. 2011;32(5):456–69.

    Article  PubMed  Google Scholar 

  180. Caponetti G, et al. Kaposi sarcoma of the musculoskeletal system. Cancer. 2007;109:1040–52.

    Article  PubMed  Google Scholar 

  181. Restrepo CS, et al. Imaging findings in musculoskeletal system: a review of 66 patients. Radiographics. 2004;24:1029–49.

    Article  PubMed  Google Scholar 

  182. Restrepo CS, Martínez S, Lemo JA MD, Carrillo JA, Lemos DF, Ojeda P, Koshy P. Imaging manifestations of Kaposi sarcoma. RadioGraphics. 2006;26:1169–85.

    Article  PubMed  Google Scholar 

  183. Nasti G, et al. Impact of highly active antiretroviral therapy on the presenting features and outcome of patients with acquired immunodeficiency syndrome-related Kaposi sarcoma. Cancer. 2003;98:2440–6.

    Article  PubMed  Google Scholar 

  184. Hiatt K, et al. Classic Kaposi sarcoma in the United States and over the last two decades: a clinicopathologic and molecular study of 438 non-HIV-related Kaposi sarcoma patients with comparison to HIV-related Kaposi Sarcoma. Mod Pathol. 2008;21(5):572–82.

    Article  CAS  PubMed  Google Scholar 

  185. Stebbing J, Portsmouth S, Gazzard B. How does HAART lead to the resolution of Kaposi’s sarcoma? Antimicrob Chemother. 2003;51:1095–8.

    Article  CAS  Google Scholar 

  186. Ledergerber B, Telenti A, Egger M. Risk of HIV-related Kaposi’s sarcoma and non-Hodgkin’s lymphoma with potent antiretroviral therapy: prospective cohort study. Swiss HIV Cohort Study. BMJ. 1999;319:23–4.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  187. Gallafent JH, Buskin S, De Turk PB, Aboulafia DM. Profile of patients with Kaposi’s sarcoma in the era of highly active antiretroviral therapy. J Clin Oncol. 2005;23:1253–60.

    Article  PubMed  Google Scholar 

  188. Cheung T. AIDS-related cancer in the era of highly active antiretroviral therapy (HAART): a model of the interplay of the immune system, virus, and cancer. “On the offensive – the Trojan Horse is being destroyed” – part A: Kaposi’s sarcoma. Cancer Invest. 2004;22(5):774–86.

    Article  CAS  PubMed  Google Scholar 

  189. Le Bourgeois JP, Frikha H, Piedbois P, Le Pechoux C, Martin L, Haddad E. Radiotherapy in the management of epidemic Kaposi’s sarcoma of the oral cavity, the eyelid, and the genitals. Radiother Oncol. 1994;30(3):263–6.

    Article  PubMed  Google Scholar 

  190. Fairley J, et al. Topical imiquimod 5% as a treatment for localized genital Kaposi’s sarcoma in an HIV-negative man: a case report. Int J STD AIDS. 2012;23(12):907–8.

    Article  CAS  PubMed  Google Scholar 

  191. Milan-Rodriguez F, Montilleó-González M, Rosales-Bordes A, Segarra-Tomas J, Salvador-Bayarri J. Kaposi’s sarcoma of the urethral meatus: management by urethral dilation. Br J Urol. 1995;75(4):559.

    Article  Google Scholar 

  192. Real F, Oettgen H, Krown S. Kaposi’s sarcoma and the acquired immunodeficiency syndrome: treatment with high and low doses of recombinant leukocyte A interferon. J Clin Oncol. 1986;4:544–51.

    CAS  PubMed  Google Scholar 

  193. Groopman J, Gottlieb M, Goodman J. Recombinant alpha-2-interferon therapy for Kaposi’s sarcoma associated with the acquired immunodeficiency syndrome. Ann Intern Med. 1984;100:671–6.

    Article  CAS  PubMed  Google Scholar 

  194. Little R, Pluda J, Wyvill K. Activity of subcutaneous interleukin-12 in AIDS-related Kaposi sarcoma. Blood. 2006;107:4650–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

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Maciolek, K.A., Abel, E.J., Jarrard, D.F., Downs, T.M. (2016). Kaposi’s Sarcoma of the Penis and Scrotum. In: Pagliaro, L. (eds) Rare Genitourinary Tumors. Springer, Cham. https://doi.org/10.1007/978-3-319-30046-7_22

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