Background: Kaposi sarcoma-associated herpesvirus (KSHV), or human herpesvirus type 8 (HHV8), is consistently identified in 2 human immunodeficiency virus (HIV)-associated lymphoproliferative disorders: primary effusion lymphoma (PEL) and multicentric Castleman disease. Rarely, KSHV/HHV8-positive extracavitary solid tissue lymphomas occur, often at extranodal sites, which are not associated with an effusion. These solid variants of PEL are similar morphologically, immunophenotypically, clinically and genetically to classic PEL. Case Report: Here we report a case of a 46-year-old HIV-positive patient with lymphadenopathy and Kaposi sarcoma of the skin. The lymph node biopsy shows a KSHV/HHV8-positive high-grade B-cell lymphoma with co-infection with Epstein-Barr virus, which supports the diagnosis of a solid variant of PEL. The same lymph node is also multifocally involved in Kaposi sarcoma. Conclusion: Analysis of viral infection is of primary importance to define this solid variant of PEL. The exact oncogenic mechanisms of HHV8 are not clearly defined. It is believed that it is related to a few latent viral gene products including ORF73, ORF72, and ORF71 which increase proliferation and impair apoptosis through variant regulatory processes. To the best of our knowledge, this is the first reported case of solid tissue PEL along with Kaposi sarcoma involving the same anatomic site.

1.
Cesarman E, Knowles DM: The role of Kaposi’s sarcoma-associated herpesvirus (KSHV/HHV-8) in lymphoproliferative diseases. Semin Cancer Biol 1999;9:165–174.
2.
Chadburn A, Hyjek E, Mathew S, Cesarman E, Said J, Knowles DM: KSHV-positive solid lymphomas represent an extra-cavitary variant of primary effusion lymphoma. Am J Surg Pathol 2004;28:1401–1416.
3.
Carbone A, Gloghini A, Vaccher E, Cerri M, Gaidano G, Dalla-Favera R, Tirelli U: Kaposi’s sarcoma-associated herpesvirus/human herpesvirus type 8-positive solid lymphomas: a tissue-based variant of primary effusion lymphoma. J Mol Diagn 2005;7:17–27.
4.
Capello D, Cerri M, Muti G, Berra E, Oreste P, Deambrogi C, Rossi D, Dotti G, Conconi A, Viganò M, Magrini U, Ippoliti G, Morra E, Gloghini A, Rambaldi A, Paulli M, Carbone A, Gaidano G: Molecular histogenesis of posttransplantation lymphoproliferative disorders. Blood 2003;102:3775–3785.
5.
DePond W, Said JW, Tasaka T, de Vos S, Kahn D, Cesarman E, Knowles DM, Koeffler HP: Kaposi’s sarcoma-associated herpesvirus and human herpesvirus 8 (KSHV/HHV8)-associated lymphoma of the bowel. Report of two cases in HIV-positive men with secondary effusion lymphomas. Am J Surg Pathol 1997;21:719–724.
6.
Carbone A, Gloghini A, Vaccher E, Zagonel V, Pastore C, Dalla Palma P, Branz F, Saglio G, Volpe R, Tirelli U, Gaidano G: Kaposi’s sarcoma-associated herpesvirus DNA sequences in AIDS-related and AIDS-unrelated lymphomatous effusions. Br J Haematol 1996;94:533–543.
7.
Said W, Chien K, Takeuchi S, Tasaka T, Asou H, Cho SK, de Vos S, Cesarman E, Knowles DM, Koeffler HP: Kaposi’s sarcoma-associated herpesvirus (KSHV or HHV8) in primary effusion lymphoma: ultrastructural demonstration of herpesvirus in lymphoma cells. Blood 1996;87:4937–4943.
8.
Young LS, Rickinson AB: Epstein-Barr virus: 40 years on. Nat Rev Cancer 2004;4:757–768.
9.
Carbone A, Cesarman E, Spina M, Gloghini A, Schulz TF: HIV-associated lymphomas and gamma-herpesviruses. Blood 2009;113:1213–1224.
10.
Du MQ, Bacon CM, Isaacson PG: Kaposi sarcoma-associated herpesvirus/human herpesvirus 8 and lymphoproliferative disorders. J Clin Pathol 2007;60:1350–1357.
11.
Verma SC, Lan K, Robertson E: Structure and function of latency-associated nuclear antigen. Curr Top Microbiol Immunol 2007;312:101–136.
12.
Dittmer D, Lagunoff M, Renne R, Staskus K, Haase A, Ganem D: A cluster of latently expressed genes in Kaposi’s sarcoma-associated herpesvirus. J Virol 1998;72:8309–8315.
13.
Petre CE, Sin SH, Dittmer DP: Functional p53 signaling in Kaposi’s sarcoma-associated herpesvirus lymphomas: implications for therapy. J Virol 2007;81:1912–1922.
14.
Klein U, Gloghini A, Gaidano G, Chadburn A, Cesarman E, Dalla-Favera R, Carbone A: Gene expression profile analysis of AIDS-related primary effusion lymphoma (PEL) suggests a plasmablastic derivation and identifies PEL-specific transcripts. Blood 2003;101:4115–4121.
15.
Gaidano G, Capello D, Fassone L, Gloghini A, Cilia AM, Ariatti C, Buonaiuto D, Vivenza D, Gallicchio M, Avanzi GC, Prat M, Carbone A: Molecular characterization of HHV-8 positive primary effusion lymphoma reveals pathogenetic and histogenetic features of the disease. J Clin Virol 2000;16:215–224.
16.
Katano H, Hoshino Y, Morishita Y, Nakamura T, Satoh H, Iwamoto A, Herndier B, Mori S: Establishing and characterizing a CD30-positive cell line harboring HHV-8 from a primary effusion lymphoma. J Med Virol 1999;58:394–401.
17.
Wilson KS, McKenna RW, Kroft SH, Dawson DB, Ansari Q, Schneider NR: Primary effusion lymphomas exhibit complex and recurrent cytogenetic abnormalities. Br J Haematol 2002;116:113–121.
18.
Carbone A, Cilia AM, Gloghini A, Capello D, Todesco M, Quattrone S, Volpe R, Gaidano G: Establishment and characterization of EBV-positive and EBV-negative primary effusion lymphoma cell lines harbouring human herpesvirus type-8. Br J Haematol 1998;102:1081–1089.
19.
Nador RG, Cesarman E, Chadburn A, Dawson DB, Ansari MQ, Sald J, Knowles DM: Primary effusion lymphoma: a distinct clinicopathologic entity associated with the Kaposi’s sarcoma-associated herpes virus. Blood 1996;88:645–656.
20.
Ghosh SK, Wood C, Boise LH, Mian AM, Deyev VV, Feuer G, Toomey NL, Shank NC, Cabral L, Barber GN, Harrington WJ Jr: Potentiation of TRAIL-induced apoptosis in primary effusion lymphoma through azidothymidine-mediated inhibition of NF-kappa B. Blood 2003;101:2321–2327.
21.
Engels EA, Pittaluga S, Whitby D, Rabkin C, Aoki Y, Jaffe ES, Goedert JJ: Immunoblastic lymphoma in persons with AIDS-associated Kaposi’s sarcoma: a role for Kaposi’s sarcoma-associated herpesvirus. Mod Pathol 2003;16:424–429.
22.
Licci S, D’Antonio A, Boscaino A, Morelli L, Piscioli F, Abbate I, Donnorso RP, Del Nonno F: Non-Hodgkin lymphomas concurrent with HHV8-associated Kaposi’s sarcoma in the same lymph node in AIDS and non-AIDS patients. Acta Haematol 2007;118:47–52.
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