A Series of Patients with Kaposi Sarcoma (Mediterranean/Classical Type): Case Presentations and Short Update on Pathogenesis and Treatment


  • Ivanka Temelkova Medical Institute of Ministry of Interior (MVR), Department of Dermatology, Venereology and Dermatologic Surgery, General Skobelev Nr 79, Sofia
  • Michael Tronnier Helios Klinikum GMBH-Dermatology, Venereology and Allergology Senator-Braun-Allee 33 Hildesheim, Hildesheim
  • Ivan Terziev Universitetska Mnogoprofilno Bolnitsa za Aktivno Lechenie "Tsaritsa Yoanna"-Common and Clinical Pathology, Sofia
  • Uwe Wollina Städtisches Klinikum Dresden-Department of Dermatology and Allergology, Friedrichstrasse 41, Dresden
  • Ilia Lozev Medical Institute of the Ministry of Interior-Surgery, Sofia
  • Mohamad Goldust Department of Dermatology, Mazandaran University of Medical Sciences, Sari
  • Georgi Tchernev Medical Institute of Ministry of Interior (MVR), Department of Dermatology, Venereology and Dermatologic Surgery, General Skobelev Nr 79, Sofia, Bulgaria; Onkoderma-Policlinic for Dermatology and Dermatologic Surgery, General Skobelev 26, Sofia




Kaposi, Pseudo-Kaposi, Stewart Treves syndrome, Radiation therapy, Treatment choice, Morbus Mali


BACKGROUND: Kaposi’s sarcoma was first described in 1872 by Moritz Kaposi. To date, it is considered a malignant disease is originating from the endothelial cells of the lymphatic vessels believed to be infected with HHV-8. The current classification defines four major epidemiological forms of Kaposi’s sarcoma: classical, endemic, AIDS-associated, and iatrogenic.

CASE REPORT: A 90-year-old male is presented with multiple plaques- and tumour-shaped brown-violet formations located on an erythematous-livid base in the area of both feet and both shanks. Two samples were taken from the lesions on the skin of the shanks, with histopathological examination and the subsequent immunohistochemistry showing Kaposi’s sarcoma.

CONCLUSIONS: Kaposi sarcoma is a disease that causes difficulties both in diagnostic and therapeutic respect. The only sure way to determine the correct diagnosis is immunohistochemical staining with the anti-HHV8 antibody. Despite the wide range of systematic and local treatment options, there is still no unified algorithm and a unified strategy for the treatment of Kaposi’s sarcoma.


Download data is not yet available.


Metrics Loading ...

Plum Analytics Artifact Widget Block


Ojala P, Schulz T. Manipulation of endothelial cells by KSHV: implications for angiogenesis and aberrant vascular differentiation. Semin Cancer Biol. 2014; 26:69-77. https://doi.org/10.1016/j.semcancer.2014.01.008 PMid:24486643

Beral V. Epidemiology of Kaposi's sarcoma. Cancer Surv. 1991; 10:5-22. PMid:1821323

O'Connell K. Kaposi's sarcoma: histopathological study of 159 cases from Malawi. J Clin Path. 1977; 30:687-695. https://doi.org/10.1136/jcp.30.8.687 PMid:599182

Pantanowitz L, Otis C, Dezube B. Immunohistochemistry in Kaposi's sarcoma. Clin Exp Dermatol. 2010; 35(1):68-72. https://doi.org/10.1111/j.1365-2230.2009.03707.x PMid:19874352

Cattelan A, Trevenzoli M, Aversa S. Recent advances in the treatment of AIDS-related Kaposi's sarcoma. Am J Clin Dermatol. 2002; 3(7):451-62. https://doi.org/10.2165/00128071-200203070-00002 PMid:12180893

Shiels R. A history of Kaposi's sarcoma. J R Soc Med. 1986; 79(9):532–534. https://doi.org/10.1177/014107688607900910 PMid:3534266 PMCid:PMC1290459

Karamanou M, Antoniou C, Stratigos A, Saridaki Z, Androutsos G. The eminent dermatologist Moriz Kaposi (1837-1902) and the first description of idiopathic multiple pigmented sarcoma of the skin. J BUON. 2013; 18(4):1101-5. PMid:24344048

Sternbach G, Varon J. Moritz Kaposi: idiopathic pigmented sarcoma of the skin. J Emerg Med. 1995; 13(5):671-4. https://doi.org/10.1016/0736-4679(95)00077-N

Fatahzadeh M. Kaposi sarcoma: review and medical management update. Oral Surg Oral Med Oral Pathol Oral Radiol. 2012; 113(1):2-16. https://doi.org/10.1016/j.tripleo.2011.05.011 PMid:22677687

Radu O, Pantanowitz L. Kaposi sarcoma. Arch Pathol Lab Med. 2013; 137(2):289-94. https://doi.org/10.5858/arpa.2012-0101-RS PMid:23368874

O'Donnell P, Pantanowitz L, Grayson W. Unique histologic variants of cutaneous Kaposi sarcoma. Am J Dermatopathol. 2010; 32(3):244-50. https://doi.org/10.1097/DAD.0b013e3181b7f6a7

Gutierrez KD, Morris VA, Wu D, Barcy S, Lagunoff M. Ets-1 is required for the activation of VEGFR3 during latent Kaposi's sarcoma-associated herpesvirus infection of endothelial cells. J Virol. 2013; 87(12):6758-68. https://doi.org/10.1128/JVI.03241-12 PMid:23552426 PMCid:PMC3676105

Berman M, Nalesnik M, Kapadia S, Rinaldo C, Jensen F. Primary lymphadenopathic Kaposi's sarcoma in an immunocompetent 23-year-old man. Am J Clin Pathol. 1986; 86(3):366-9. https://doi.org/10.1093/ajcp/86.3.366 PMid:3752000

Hirschel B, Wyss M, Thélin O, Cox J. Lymphadenopathic form of Kaposi's sarcoma (KS) in an African child. No evidence for acquired immunodeficiency syndrome (AIDS). Helv Paediatr Acta. 1985; 40(6):481-4. PMid:3007397

David O, Sani I, Azuhq P, Samaila M, Shehu M, Abdullah A. Lymphadenopathic Kaposi's sarcoma in an immunocompetent adult. J Coll Physicians Surg Pak. 2012; 22(6):403-5. PMid:22630105

Castelli E, Wollina U. Histopathologic features of progression in Mediterranean and immunodeficiency-related Kaposi sarcoma. Am J Dermatopathol. 2000; 22(1):89-91. https://doi.org/10.1097/00000372-200002000-00019 PMid:10698226

Wahman A, Melnick S, Rhame F, Potter J. The epidemiology of classic, African, and immunosuppressed Kaposi's sarcoma. Epidemiol Rev. 1991; 13:178-99. https://doi.org/10.1093/oxfordjournals.epirev.a036068 PMid:1765111

Mehta S, Garg A, Gupta L, Mittal A, Khare A, Kuldeep C. Kaposi's sarcoma as a presenting manifestation of HIV. Indian J Sex Transm Dis AIDS. 2011; 32(2):108–110. https://doi.org/10.4103/0253-7184.85415 PMid:22021973 PMCid:PMC3195171

Cahoon E, Linet M, Clarke C, Pawlish K, Engels E, Pfeiffer R. Risk of Kaposi sarcoma after solid organ transplantation in the United States. Int J Cancer. 2018. https://doi.org/10.1002/ijc.31735 PMid:29987894

Blumenfeld W, Egbert B, Sagebiel R. Differential diagnosis of Kaposi's sarcoma. Arch Pathol Lab Med. 1985; 109(2):123-7. PMid:2983633

Pantanowitz L, Dezube B. Kaposi sarcoma in unusual locations. BMC Cancer. 2008; 8:190. https://doi.org/10.1186/1471-2407-8-190 PMid:18605999 PMCid:PMC2464778

Rosado F, Itani D, Coffin C, Cates J. 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(3):301-4. https://doi.org/10.5858/arpa.2011-0213-OA PMid:22372906

Audard V, Lok C, Trabattoni M, Wechsler J, Brousse N, Fraitag S. [Misleading Kaposi's sarcoma: usefulness of anti HHV-8 immunostaining]. Ann Pathol. 2003; 23(4):345-8. PMid:14597899

Salameire D, Templier I, Charles J, Pinel N, Morand P, Leccia M, Lantuejoul S. An "anaplastic" Kaposi's sarcoma mimicking a Stewart-Treves syndrome. A case report and a review of literature. Am J Dermatopathol. 2008; 30(3):265-8. https://doi.org/10.1097/DAD.0b013e318169fd5f PMid:18496429

Tchernev G, Yungareva I, Mangarov H, Stavrov K, Lozev I, Temelkova I, Chernin S, Pidakev I, Tronnier M. Pretibial Located Stewart-Treves Syndrome: Uncommon Presentation in a Bulgarian Patient!. Open Access Maced J Med Sci. 2018; 6(4):663–665. https://doi.org/10.3889/oamjms.2018.191 PMid:29731936 PMCid:PMC5927499

Tchernev G, Patterson J, Ananiev J, Tronnier M. Unilateral presentation of pseudo-Kaposi's acroangiodermatitis--a diagnostic and therapeutic challenge. Vojnosanit Pregl. 2012; 69(4):370-3. https://doi.org/10.2298/VSP1204370T PMid:22624432

Wollina U. Angiosarcoma: an immunogenic tumour. Br J Dermatol. 2018; 179, pp239–259.

Harris BHL, Walsh JL, Neciunaite R et al. Ring a ring oʼroses, a patient with Kaposiʼs? Pazopanib, pazopanib, it might go away. Mediterranean(classic) Kaposi sarcoma responds to the tyrosine kinase inhibitor pazopanib after multiple lines of standard therapy. Clin Exp Dermatol. 2018; 43:234-236. https://doi.org/10.1111/ced.13302 PMid:29282751

Sullivan R, Pantanowitz L. New drug targets in Kaposi sarcoma. Expert Opin Ther Targets. 2010; 14(12):1355-66. https://doi.org/10.1517/14728222.2010.532336 PMid:21043836

Geara F, Le Bourgeois P, Piedbois P, Pavlovitch M, Mazeron J. Radiotherapy in the management of cutaneous epidemic Kaposi's sarcoma. Int J Radiat Oncol Biol Phys. 1991; 21(6):1517-22. https://doi.org/10.1016/0360-3016(91)90327-Z

Ramírez K, Zavala J, Morán D, Hernández D, Jiménez A. Classic Kaposi's sarcoma - complete response to radiation therapy: a case report. J Med Case Rep. 2016; 10:322. https://doi.org/10.1186/s13256-016-1101-6 PMid:27832802 PMCid:PMC5105307

Donato V, Guarnaccia R, Dognini J, de Pascalis G, Caruso C, Bellagamba R, Morrone A. Radiation therapy in the treatment of HIV-related Kaposi's sarcoma. Anticancer Res. 2013; 33(5):2153-7. PMid:23645769

Piedbois P, Frikha H, Martin L, Levy E, Haddad E, Le Bourgeois J. Radiotherapy in the management of epidemic Kaposi's sarcoma. Int J Radiat Oncol Biol Phys. 1994; 30(5):1207-11. https://doi.org/10.1016/0360-3016(94)90330-1

Chang J, Kim I. Role of radiotherapy in local control of non-AIDS associated Kaposi's sarcoma patients in Korea: a single institution experience. Radiat Oncol J. 2012; 30(4):153–157. https://doi.org/10.3857/roj.2012.30.4.153 PMid:23346533 PMCid:PMC3546282

Aboulafia D. Regression of acquired immunodeficiency syndrome-related pulmonary Kaposi's sarcoma after highly active antiretroviral therapy. Mayo Clin Proc. 1998; 73(5):439-43. https://doi.org/10.1016/S0025-6196(11)63726-9

Nagy S, Gyulai R, Kemeny L, Szenohradszky P, Dobozy A. Iatrogenic Kaposi's sarcoma: HHV8 positivity persists but the tumors regress almost completely without immunosuppressive therapy. Transplantation. 2000; 69(10):2230-1. https://doi.org/10.1097/00007890-200005270-00053 PMid:10852635

Vincenzi B, D'Onofrio L, Frezza A, Grasso R, Fausti V, Santini D, Tos A, Tonini G. Classic Kaposi Sarcoma: to treat or not to treat? BMC Res Notes. 2015; 8:138. https://doi.org/10.1186/s13104-015-1076-1 PMid:25889316 PMCid:PMC4395989

WHO. Guidelines on the Treatment of Skin and Oral HIV-Associated Conditions in Children and Adults. Geneva: World Health Organization, 2014.



How to Cite

Temelkova I, Tronnier M, Terziev I, Wollina U, Lozev I, Goldust M, Tchernev G. A Series of Patients with Kaposi Sarcoma (Mediterranean/Classical Type): Case Presentations and Short Update on Pathogenesis and Treatment. Open Access Maced J Med Sci [Internet]. 2018 Aug. 20 [cited 2023 Dec. 6];6(9):1688-93. Available from: https://oamjms.eu/index.php/mjms/article/view/oamjms.2018.354



C- Case Reports

Most read articles by the same author(s)

1 2 3 4 5 6 7 8 9 10 > >>