Kaposi Sarkomu
Özet
Kaposi sarkomu (KS), Human Herpes Virüs 8 (HHV-8) enfeksiyonu sonucunda gelişen ve endotel kaynaklı anjiyoproliferatif özellik gösteren malign vasküler bir tümördür. Tıbbi literatürde epidemiyolojik ve klinik özelliklerine göre immünsupresyon ilişkili, HIV ilişkili, klasik (Akdeniz) ve Afrika endemik olmak üzere dört ana alt tipte sınıflandırılır. Hastalık sıklıkla deri ve mukoza yüzeylerinde mor veya kırmızı renkli değişken boyutlu lezyonlarla karakterize olsa da, lenf nodları ve hayati iç organ tutulumları nedeniyle ölümcül tablolara yol açabilir. İmmünsupresyon ilişkili alt tip özellikle organ transplantasyonu sonrasında yoğun immünsupresif tedavi alan hastalarda gözlenirken, HIV ilişkili alt tip antiretroviral tedaviler öncesinde AIDS hastalarında oldukça yaygın ve agresif seyretmiştir. Klasik tip genellikle Akdeniz kökenli yaşlı erkeklerde yavaş seyirli (indolent) bir klinik takip ederken, Afrika endemik tipi alt ekstremiteleri tutan daha saldırgan bir doğaya sahiptir. Kaposi sarkomunun multidisipliner tedavi yönetimi tümörün tipine, evresine ve hastanın performans statüsüne göre belirlenir. Hafif seyirli vakalarda izlem ve lokal cerrahi, kriyoterapi veya radyoterapi gibi bölgesel palyatif yöntemler tercih edilirken; yaygın, semptomatik ve viseral tutulumu olan agresif olgularda lipozomal doksorubisin ve paklitaksel gibi ajanlarla sistemik kemoterapi protokolleri başarıyla uygulanmaktadır.
Kaposi sarcoma (KS) is a malignant vascular tumor caused by Human Herpesvirus 8 (HHV-8) infection, characterized by angioproliferative endothelial proliferation. The disease is classified into four distinct clinicopathological subtypes based on epidemiological features: immunosuppression-associated, HIV-associated, classic, and African endemic Kaposi sarcoma. Although KS typically presents as variable-sized purple or red mucocutaneous lesions on the skin and mucous membranes, it can also involve the lymph nodes and visceral organs, leading to life-threatening complications. The immunosuppression-associated subtype is predominantly observed in solid organ transplant recipients under intensive immunosuppressive regimens, whereas the HIV-associated form was highly prevalent and aggressive among AIDS patients prior to the widespread use of antiretroviral therapies. The classic form usually presents as an indolent disease in elderly men of Mediterranean descent, whereas the African endemic type exhibits a more aggressive clinical course, primarily targeting the lower extremities in Equatorial African countries. The therapeutic management of Kaposi sarcoma is strictly individualized based on the specific subtype, tumor extent, and the patient's overall performance status. While asymptomatic cosmetic lesions can be managed with active surveillance, localized interventions such as surgery, cryotherapy, or radiation therapy are preferred for symptomatic local diseases. For advanced, symptomatic, or visceral cases, systemic chemotherapy regimens utilizing agents like liposomal doxorubicin and paclitaxel remain the standard of care, with alternative chemotherapeutic or immunomodulatory agents reserved for refractory cases.
Referanslar
Cesarman E, Damania B, Krown SE, et al. Kaposi sarcoma. Nat Rev Dis Primers. 2019 Jan 31;5(1):9. doi: 10.1038/s41572-019-0060-9. PMID: 30705286; PMCID: PMC6685213.
Grabar S, Costagliola D. Epidemiology of Kaposi's Sarcoma. Cancers (Basel). 2021 Nov 14;13(22):5692. doi: 10.3390/cancers13225692. PMID: 34830846; PMCID: PMC8616388.
Etemad SA, Dewan AK. Kaposi Sarcoma Updates. Dermatol Clin. 2019 Oct;37(4):505-517. doi: 10.1016/j.det.2019.05.008. Epub 2019 Jul 10. PMID: 31466590.
Radu O, Pantanowitz L. Kaposi sarcoma. Arch Pathol Lab Med. 2013 Feb;137(2):289-94. doi: 10.5858/arpa.2012-0101-RS. PMID: 23368874.
Francès C, Lebbé C. Kaposi's sarcoma. Cancer Treat Res. 2009;146:299-309. doi: 10.1007/978-0-387-78574-5_24. PMID: 19415211.
Mbulaiteye SM, Engels EA. Kaposi's sarcoma risk among transplant recipients in the United States (1993-2003). Int J Cancer. 2006 Dec 1;119(11):2685-91. doi: 10.1002/ijc.22233. PMID: 16929513.
Hoffmann C, Sabranski M, Esser S. HIV-Associated Kaposi's Sarcoma. Oncol Res Treat. 2017;40(3):94-98. doi: 10.1159/000455971. Epub 2017 Feb 9. PMID: 28259888.
Yarchoan R, Uldrick TS. HIV-Associated Cancers and Related Diseases. N Engl J Med. 2018 Mar 15;378(11):1029-1041. doi: 10.1056/NEJMra1615896. PMID: 29539283; PMCID: PMC6890231.
Dalla Pria A, Pinato DJ, Bracchi M, et al. Recent advances in HIV-associated Kaposi sarcoma. F1000Res. 2019 Jun 26;8:F1000 Faculty Rev-970. doi: 10.12688/f1000research.17401.1. PMID: 31297181; PMCID: PMC6600854.
Brambilla L, Genovese G, Berti E, et al. Diagnosis and treatment of classic and iatrogenic Kaposi's sarcoma: Italian recommendations. Ital J Dermatol Venerol. 2021 Jun;156(3):356-365. doi: 10.23736/S2784-8671.20.06703-6. Epub 2020 Nov 12. PMID: 33179877.
Simonart T. Role of environmental factors in the pathogenesis of classic and African-endemic Kaposi sarcoma. Cancer Lett. 2006 Nov 28;244(1):1-7. doi: 10.1016/j.canlet.2006.02.005. Epub 2006 Mar 20. PMID: 16542773.
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 May;33(5):2153-7. PMID: 23645769.
Kutlubay Z, Küçüktaş M, Yardımcı G, Engin B, Serdaroğlu S. Evaluation of effectiveness of cryotherapy on the treatment of cutaneous Kaposi's sarcoma. Dermatol Surg. 2013 Oct;39(10):1502-6. doi: 10.1111/dsu.12285. Epub 2013 Jul 23. PMID: 23879208.
Cianfrocca M, Lee S, Von Roenn J, et al. Randomized trial of paclitaxel versus pegylated liposomal doxorubicin for advanced human immunodeficiency virus-associated Kaposi sarcoma: evidence of symptom palliation from chemotherapy. Cancer. 2010 Aug 15;116(16):3969-77. doi: 10.1002/cncr.25362. PMID: 20564162; PMCID: PMC3157242.