GUIDELINES IN DERMATOLOGY

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KAPOSI’S SARCOMA

Emel Bülbül Başkan1
Ezgi Akın2

1Bursa Uludağ University, Faculty of Medicine, Department of Dermatology, Bursa, Türkiye
2Bursa Uludağ University, Faculty of Medicine, Department of Dermatology, Bursa, Türkiye

Bülbül Başkan E, Akın E. Kaposi’s Sarcoma. In: Kutlubay Z, editor. Guidelines in Dermatology. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.103-110.

ABSTRACT

The rare, malignant, multilocular vascular diseases known as Kaposi’s sarcoma (KS) is caused by lymphatic endothelial cells and primarily affects the skin and mucous membranes, although it can also affect internal organs such the liver, lungs, and gastrointestinal tract as well as the lymphatic system. Pathogenesis is characterized by aberrant neoangiogenesis, inflammation, and proliferation of lymphoendothelial tumor cells. Clinical features serve as the main basis for the diagnosis, which is then confirmed by histology and immunohistochemistry. The type of KS determines the distribution and intensity of cutaneous involvement as well as mucosal, nodal, and visceral involvement. The classic subtype has a chronic progression and generally indolent behavior, while the iatrogenic or epidemic subtypes have more severe manifestations. Although skin lesions can appear anywhere, they are most commonly found in the head, neck, and lower limbs. According to guidelines in laboratory examination when KS is first diagnosed, HIV testing should be made available to all patients who are not known to be infected If HIV is detected or in iatrogenic KS, cellular immune status should be evaluated. The histopathology of Kaposi sarcoma includes proliferation, inflammation and angiogenesis. Some tests can be used for staging during diagnosis. These include endoscopy, colonoscopy, whole body computed tomography and bronchoscopy. Most frequently advanced age is one of the poor prognostic factors for Kaposi’s sarcoma. There are different prognostic factors for different subtypes. There isn’t a standard therapy regimen that is approved by all for the management of KS. There are systemic and local therapies for KS treatment. Local treatment included excision, cryotherapy, radiation, camouflage, intralesional injections of chemotherapeutic agents, topical therapies, and different modalities that can be used in combination. The local treatment choice depends on size, localization and morphology of the lesion. For small macular or nodular lesions cryotherapy, excision, intralesional vincristine, vinblastine, interferons; alitretinoin gel, imiquimod, cauterization with silver nitrate can be used. For all KS subtypes, radiatiotherapy is one of the most successful local therapies because of KS’s great radiosensitivity. Different approaches are mentioned for systemic therapy. Pegylated liposomal doxorubicin, anthracycline, bleomycin, vincristine, vinblastine, etoposide, paclitaxel, gemcitabine and pomalidomide are suggested options for systemic treatment. In this review, dermatological approach to Kaposi Sarcoma has been summarized based on current guidelines.

Keywords: Kaposi’s Sarcoma; AIDS-related Kaposi sarcoma; Guideline; Diagnosis; Treatment; Herpesvirus 8

Referanslar

  1. Antman K, Chang Y. Kaposi's sarcoma. N Engl J Med. 2000;342:1027e38. [Crossref]  [PubMed]
  2. Esser S, Schöfer H, Hoffmann C, Claßen J, Kreuter A, Leiter U, et al. S1 Guidelines for the Kaposi Sarcoma. J Dtsch Dermatol Ges. 2022;20(6):892-904. [Crossref]
  3. Grulich AE, Beral V, Swerdlow AJ. Kaposi's sarcoma in England and Wales before the AIDS epidemic. Br J Cancer. 1992;66:1135e7. [Crossref]  [PubMed]  [PMC]
  4. Denis D, Seta V, Regnier-Rosencher E, Kramkimel N, Chanal J, Avril MF, et al. A fifth subtype of Kaposi's sarcoma, classic Kaposi's sarcoma in men who have sex with men: a cohort study in Paris. J Eur Acad Dermatol Venereol. 2018;32(8): 1377-1384. [Crossref]  [PubMed]
  5. 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(5):239-50. [Crossref]  [PubMed]
  6. Lebbe C, Legendre C, Frances C. Kaposi sarcoma in transplantation. Transplant Rev (Orlando). 2008;22:252-61. [Crossref]  [PubMed]
  7. Hoffmann C, Sabranski M, Esser S. HIV-Associated Kaposi's Sarcoma. Oncol Res Treat 2017;40(3):94-98 [Crossref]  [PubMed]
  8. Schneider JW, Dittmer DP. Diagnosis and Treatment of Kaposi Sarcoma. Am J Clin Dermatol 2017;18:529-39. [Crossref]  [PubMed]  [PMC]
  9. Ertürk Yılmaz T, Akay BN, Okçu Heper A. Dermoscopic findings of Kaposi sarcoma and dermatopathological correlations. Australas J Dermatol. 2020;61:e46-53. [Crossref]  [PubMed]
  10. Mitsuyasu RT. Clinical variants and staging of Kaposi's sarcoma. Semin Oncol. 1987;14 Suppl.3:13-8. [PubMed]
  11. Lebbe C, Garbe C, Stratigos AJ, et al. Diagnosis and treatment of Kaposi's sarcoma: European consensus-based interdisciplinary guideline (EDF/EADO/EORTC). Eur J Cancer. 2019;114:117-127. [Crossref]  [PubMed]
  12. Bower M, Palfreeman A, Alfa-Wali M, Bunker C, Burns F, Churchill D, et al.; British HIV Association. British HIV Association guidelines for HIV-associated malignancies 2014. HIV Med. 2014;15(Suppl2):1-92. [Crossref]  [PubMed]
  13. Whitby D, Howard MR, Tenant-Flowers M, Brink NS, Copas A, Boshoff C et al. Detection of Kaposi sarcoma associated herpesvirus in peripheral blood of HIV-infected individuals and progression to Kaposi's sarcoma. Lancet. 1995;346:799802. [Crossref]  [PubMed]
  14. Brambilla L, Genovese G, Berti E, Peris K, Rongioletti F, Micali G, et al. Diagnosis and treatment of classic and iatrogenic Kaposi's sarcoma: Italian recommendations. Ital J Dermatol Venerol. 2021;156(3):356-365. [Crossref]  [PubMed]
  15. Krown SE, Testa MA, Huang J. AIDS-related Kaposi's sarcoma: prospective validation of the AIDS Clinical Trials Group staging classification. AIDS Clinical Trials Group Oncology Committee. J Clin Oncol. 1997;15:3085-92. [Crossref]  [PubMed]
  16. Dezube BJ, Pantanowitz L, Aboulafia DM. Management of AIDS-related Kaposi sarcoma: advances in target discovery and treatment. AIDS Read. 2004;14:236-8,243-4,251-3. [PubMed]
  17. Régnier-Rosencher E, Guillot B, Dupin N. Treatments for classic Kaposi sarcoma: a systematic review of the literature. J Am Acad Dermatol. 2013;68(2):313-31. [Crossref]  [PubMed]
  18. Saiag P, Brunet H, Fortier-Beaulieu M. [Local treatments of AIDS-related Kaposi disease]. Ann Dermatol Venereol 1995;122:551-7 [PubMed]
  19. Tappero JW, Berger TG, Kaplan LD, Volberding PA, Kahn JO. Cryotherapy for cutaneous Kaposi's sarcoma (KS) associated with acquired immune deficiency syndrome (AIDS): a phase II trial. J Acquir Immune Defic Syndr 1991;4:839-46. [Crossref]  [PubMed]
  20. Brambilla L, Bellinvia M, Tourlaki A, Scoppio B, Gaiani F, Boneschi V. Intralesional vincristine as first-line therapy for nodular lesions in classic Kaposi sarcoma: a prospective study in 151 patients. Br J Dermatol. 2010;162:854e9. [Crossref]  [PubMed]
  21. Di Monta G, Caraco C, Benedetto L, La Padula S, Marone U, Tornesello ML, et al. Electrochemotherapy as "new standard of care" treatment for cutaneous Kaposi's sarcoma. Eur J Surg Oncol. 2014;40:61e6. [Crossref]  [PubMed]
  22. Celestin Schartz NE, Chevret S, Paz C, Kerob D, Verola O, Morel P, et al. Imiquimod 5% cream for treatment of HIV-negative Kaposi's sarcoma skin lesions: A phase I to II, open-label trial in 17 patients. J Am Acad Dermatol. 2008;58: 585-91. [Crossref]  [PubMed]
  23. Bodsworth NJ, Bloch M, Bower M, Donnell D, Yocum R, International Panretin Gel KSSG. Phase III vehicle-controlled, multi-centered study of topical alitretinoin gel 0.1% in cutaneous AIDS-related Kaposi's sarcoma. Am J Clin Dermatol. 2001;2:77-87. [Crossref]  [PubMed]
  24. Stelzer KJ, Griffin TW. A randomized prospective trial of radiation therapy for AIDS-associated Kaposi's sarcoma. Int J Radiat Oncol Biol Phys. 1993;27:1057-61. [Crossref]  [PubMed]
  25. Thariat J, Kirova Y, Sio T, Choussy O, Choussy O, Vees H, Schick U, et al. Mucosal Kaposi sarcoma, a rare cancer network study. Rare Tumors. 2012;4(4):e49. [Crossref]  [PubMed]  [PMC]
  26. Brambilla L, Labianca R, Boneschi V, Fossati S, Dallavalle G, Finzi AF, et al. Mediterranean Kaposi's sarcoma in the elderly. A randomized study of oral etoposide versus vinblastine. Cancer. 1994;74:2873-8. [Crossref]  [PubMed]
  27. Hernandez DE, Perez JR. Advanced epidemic Kaposi's sarcoma: treatment with bleomycin or combination of doxorubicin, bleomycin, and vincristine. Int J Dermatol. 1996;35:831-3. [Crossref]  [PubMed]
  28. Yarchoan R, Uldrick TS. HIV-associated cancers and related diseases. N Engl J Med. 2018;378:1029-41. [Crossref]  [PubMed]  [PMC]
  29. Krown SE, Li P, Von Roenn JH, Paredes J, Huang J, Testa MA. Efficacy of low-dose interferon with antiretroviral therapy in Kaposi's sarcoma: a randomized phase II AIDS clinical trials group study. J Interferon Cytokine Res. 2002;22:295-303. [Crossref]  [PubMed]
  30. Arruda É, Jacome AA, Toscano AL, 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 [published correction appears in Braz J Infect Dis. 2014 Jul-Aug;18(4):468]. Braz J Infect Dis. 2014;18(3):315-326. [Crossref]  [PubMed]  [PMC]