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| __NOTOC__ | | __NOTOC__ |
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| {{CMG}} {{AE}} {{HL}} | | {{CMG}} {{AE}} {{HL}} |
| {{Kaposi's sarcoma}} | | {{Kaposi's sarcoma}} |
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| ==Overview== | | ==Overview== |
| Kaposi's sarcoma arises from [[endothelial cell]]s, which are [[epithelial cell]]s that normally lines the luminal surface of [[blood vessel]]s and [[lymphatic]] vessels.<ref name="pmid23806158">{{cite journal| author=Ruocco E, Ruocco V, Tornesello ML, Gambardella A, Wolf R, Buonaguro FM| title=Kaposi's sarcoma: etiology and pathogenesis, inducing factors, causal associations, and treatments: facts and controversies. | journal=Clin Dermatol | year= 2013 | volume= 31 | issue= 4 | pages= 413-22 | pmid=23806158 | doi=10.1016/j.clindermatol.2013.01.008 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23806158 }} </ref><ref name="patho2"> Kaposi's Sarcoma. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/skintumornonmelanocytickaposisarcoma.html Accessed on January, 19 2015</ref><ref name="wiki">Kaposi's sarcoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Kaposi's_sarcoma Accessed on January, 17 2015</ref> Kaposi's sarcoma is mainly caused by an [[infection]] with Human herpes virus 8 ([[HHV-8]]), which is also known as Kaposi's sarcoma-associated herpes virus (KSHV). The main [[gene]] involved in the pathogensis of Kaposi's sarcoma is ORF73 gene, which encodes the viral latency-associated [[nuclear]] [[antigen]] (LANA-1).<ref name="pmid23698402">{{cite journal| author=Toth Z, Brulois K, Jung JU| title=The chromatin landscape of Kaposi's sarcoma-associated herpesvirus. | journal=Viruses | year= 2013 | volume= 5 | issue= 5 | pages= 1346-73 | pmid=23698402 | doi=10.3390/v5051346 | pmc=PMC3712311 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23698402 }} </ref> Kaposi's sarcoma is commonly associated with acquired immune deficiency syndrome ([[AIDS]]). On gross pathology, reddish, violaceous, or bluish-black [[macule]]s and patches are characteristic findings of Kaposi's sarcoma. On microscopic histopathological analysis the presence of [[spindle cell]]s with minimal [[nuclear]] atypia are characteristic findings of Kaposi's sarcoma.<ref name="pmid25375885">{{cite journal| author=Rossetto CC, Pari GS| title=PAN's Labyrinth: Molecular biology of Kaposi's sarcoma-associated herpesvirus (KSHV) PAN RNA, a multifunctional long noncoding RNA. | journal=Viruses | year= 2014 | volume= 6 | issue= 11 | pages= 4212-26 | pmid=25375885 | doi=10.3390/v6114212 | pmc=PMC4246217 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25375885 }} </ref><ref name="pmid23685018">{{cite journal| author=Cancian L, Hansen A, Boshoff C| title=Cellular origin of Kaposi's sarcoma and Kaposi's sarcoma-associated herpesvirus-induced cell reprogramming. | journal=Trends Cell Biol | year= 2013 | volume= 23 | issue= 9 | pages= 421-32 | pmid=23685018 | doi=10.1016/j.tcb.2013.04.001 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23685018 }} </ref><ref name="pmid25068226">{{cite journal| author=Zattra E Coati I, Alaibac M, Piaserico S| title=Kaposi's sarcoma and other rare skin cancers in organ transplant patients. | journal=G Ital Dermatol Venereol | year= 2014 | volume= 149 | issue= 4 | pages= 395-400 | pmid=25068226 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25068226 }} </ref><ref name="radio"> Kaposi's Sarcoma. Radiopaedia (2015) http://radiopaedia.org/articles/kaposi-sarcoma Accessed on January, 19 2016</ref> Kaposi's sarcoma may be classified according to the clinical setting of the disease into five subtypess: classic Kaposi's sarcoma, African cutaneous Kaposi's sarcoma, African lymphadenopathic Kaposi's sarcoma, iatrogenic Kaposi's sarcoma, and AIDS-associated Kaposi's sarcoma.<ref name="wiki">Kaposi's sarcoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Kaposi's_sarcoma Accessed on January, 17 2015</ref> The epidemiology of Kaposi sarcoma varies depending on its on the specific subtype. Before the [[AIDS]] epidimic, Kaposi's sarcoma was relatively rare. In the early epidemic of [[AIDS]], the incidence of HIV-related Kaposi sarcoma significantly increased;<ref name=cancer.gov>[http://www.cancer.org/cancer/kaposisarcoma/detailedguide/kaposi-sarcoma-what-is-key-statistics What are the key statistics about Kaposi sarcoma? Cancer.org]</ref> however, [[antiretroviral therapy]] led to a decrease in the incidence of HIV-related Kaposi sarcoma.<ref name="pmid12853764">{{cite journal| author=Portsmouth S, Stebbing J, Gill J, Mandalia S, Bower M, Nelson M et al.| title=A comparison of regimens based on non-nucleoside reverse transcriptase inhibitors or protease inhibitors in preventing Kaposi's sarcoma. | journal=AIDS | year= 2003 | volume= 17 | issue= 11 | pages= F17-22 | pmid=12853764 | doi=10.1097/01.aids.0000076309.76477.f1 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12853764 }} </ref><ref name="pmid16849755">{{cite journal| author=Grabar S, Abraham B, Mahamat A, Del Giudice P, Rosenthal E, Costagliola D| title=Differential impact of combination antiretroviral therapy in preventing Kaposi's sarcoma with and without visceral involvement. | journal=J Clin Oncol | year= 2006 | volume= 24 | issue= 21 | pages= 3408-14 | pmid=16849755 | doi=10.1200/JCO.2005.05.4072 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16849755 }} </ref> While endemic African Kaposi sarcoma is more common in African Bantu,<ref name="pmid1765111">{{cite journal| author=Wahman A, Melnick SL, Rhame FS, Potter JD| title=The epidemiology of classic, African, and immunosuppressed Kaposi's sarcoma. | journal=Epidemiol Rev | year= 1991 | volume= 13 | issue= | pages= 178-99 | pmid=1765111 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1765111 }} </ref> classic Kaposi sarcoma is more prevalent in Middle Eastern countries particularly among males and individuals from Jewish descent.<ref name="pmid1765111">{{cite journal| author=Wahman A, Melnick SL, Rhame FS, Potter JD| title=The epidemiology of classic, African, and immunosuppressed Kaposi's sarcoma. | journal=Epidemiol Rev | year= 1991 | volume= 13 | issue= | pages= 178-99 | pmid=1765111 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1765111 }} </ref><ref name="pmid8804445">{{cite journal| author=Franceschi S, Geddes M| title=Epidemiology of classic Kaposi's sarcoma, with special reference to mediterranean population. | journal=Tumori | year= 1995 | volume= 81 | issue= 5 | pages= 308-14 | pmid=8804445 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8804445 }} </ref> The incidence of Kaposi sarcoma is markedly increased in renal transplant patients ranging from 0.5% to 5.3%.<ref name="pmid17456614">{{cite journal| author=Campistol JM, Schena FP| title=Kaposi's sarcoma in renal transplant recipients--the impact of proliferation signal inhibitors. | journal=Nephrol Dial Transplant | year= 2007 | volume= 22 Suppl 1 | issue= | pages= i17-22 | pmid=17456614 | doi=10.1093/ndt/gfm089 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17456614 }} </ref> Transplant-related Kaposi sarcoma occurs more commonly in individuals who have Jewish, Arabic, Mediterranean, African, or Caribbean ethnicity.<ref name="pmid17456614">{{cite journal| author=Campistol JM, Schena FP| title=Kaposi's sarcoma in renal transplant recipients--the impact of proliferation signal inhibitors. | journal=Nephrol Dial Transplant | year= 2007 | volume= 22 Suppl 1 | issue= | pages= i17-22 | pmid=17456614 | doi=10.1093/ndt/gfm089 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17456614 }} </ref> In the United States, the age-adjusted [[prevalence]] of Kaposi sarcoma is 8.1 per 100,000 in 2011.<ref name="SEER">Howlader N, Noone AM, Krapcho M, Garshell J, Miller D, Altekruse SF, Kosary CL, Yu M, Ruhl J, Tatalovich Z,Mariotto A, Lewis DR, Chen HS, Feuer EJ, Cronin KA (eds). SEER Cancer Statistics Review, 1975-2011, National Cancer Institute. Bethesda, MD, http://seer.cancer.gov/csr/1975_2011/, based on November 2013 SEER data submission, posted to the SEER web site, April 2014.</ref> The most potent risk factor in the development of Kaposi's sarcoma is an immune deficiency state. There is no established system for the staging of Kaposi's sarcoma among [[immune]] competent patients. However, the staging of [[AIDS]]-associated Kaposi's sarcoma is based on the AIDS Clinical Trials Group (ACTG) staging system. Symptoms of Kaposi's sarcoma include [[fever]], [[abdominal pain]], [[hematochezia]], [[hemoptysis]], and gradual development of red/violaceous [[macule]]s and patches over the nose, legs, and feet.<ref name="wiki">Kaposi's sarcoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Kaposi's_sarcoma Accessed on January, 17 2015</ref><ref name="Nation">Kaposi Sarcoma Treatment–for health professionals. National Cancer Institute (2015) http://www.cancer.gov/types/soft-tissue-sarcoma/hp/kaposi-treatment-pdq#section/_15 Accessed on January, 20 2015</ref><ref name="cancer"> Sarcoma- Kaposi Guide. Cancer.Net (2015) http://www.cancer.net/cancer-types/sarcoma-kaposi/risk-factors-and-prevention Accessed on January, 20 2015</ref><ref name="pmid21963166">{{cite journal| author=Restrepo CS, Ocazionez D| title=Kaposi's sarcoma: imaging overview. | journal=Semin Ultrasound CT MR | year= 2011 | volume= 32 | issue= 5 | pages= 456-69 | pmid=21963166 | doi=10.1053/j.sult.2011.03.007 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21963166 }} </ref> The optimal therapy for Kaposi's sarcoma depends on multiple factors. Management strategies varies depending on the specific variant of Kaposi's sarcoma. Classic Kaposi's sarcoma management may range from no treatment to either [[radiotherapy]], local therapeutic interventions, or surgical excision. Iatrogenic Kaposi's sarcoma management focuses on modifying [[immunosuppressive therapy]] in addition to local therapeutic interventions. Endemic Kaposi's sarcoma is primarily manage by systemic [[chemotherapy]]. However, there is no curative treatment for epidemic Kaposi's sarcoma; the mainstay management for such patients is [[Highly active antiretroviral therapy|HAART therapy]] which aims for the control of Kaposi's sarcoma progression.<ref name="pmid22677687">{{cite journal| author=Fatahzadeh M| title=Kaposi sarcoma: review and medical management update. | journal=Oral Surg Oral Med Oral Pathol Oral Radiol | year= 2012 | volume= 113 | issue= 1 | pages= 2-16 | pmid=22677687 | doi=10.1016/j.tripleo.2011.05.011 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22677687 }} </ref><ref name="pmid24585567">{{cite journal| author=Masur H, Brooks JT, Benson CA, Holmes KK, Pau AK, Kaplan JE et al.| title=Prevention and treatment of opportunistic infections in HIV-infected adults and adolescents: Updated Guidelines from the Centers for Disease Control and Prevention, National Institutes of Health, and HIV Medicine Association of the Infectious Diseases Society of America. | journal=Clin Infect Dis | year= 2014 | volume= 58 | issue= 9 | pages= 1308-11 | pmid=24585567 | doi=10.1093/cid/ciu094 | pmc=PMC3982842 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24585567 }} </ref> Local/regional therapy recommended for the management of Kaposi's sarcoma patients may include surgical excision, [[cryotherapy]], and [[sclerotherapy]].<ref name="pmid22677687">{{cite journal| author=Fatahzadeh M| title=Kaposi sarcoma: review and medical management update. | journal=Oral Surg Oral Med Oral Pathol Oral Radiol | year= 2012 | volume= 113 | issue= 1 | pages= 2-16 | pmid=22677687 | doi=10.1016/j.tripleo.2011.05.011 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22677687 }} </ref> | | Kaposi's sarcoma arises from [[endothelial cell]]s, which are [[epithelial cell]]s that normally lines the luminal surface of [[blood vessel]]s and [[lymphatic]] vessels. Kaposi's sarcoma is mainly caused by an [[infection]] with Human herpes virus 8 ([[HHV-8]]), which is also known as Kaposi's sarcoma-associated herpes virus (KSHV). The main [[gene]] involved in the pathogensis of Kaposi's sarcoma is ORF73 gene, which encodes the viral latency-associated [[nuclear]] [[antigen]] (LANA-1). Kaposi's sarcoma is commonly associated with acquired immune deficiency syndrome ([[AIDS]]). On gross pathology, reddish, violaceous, or bluish-black [[macule]]s and patches are characteristic findings of Kaposi's sarcoma. On microscopic histopathological analysis the presence of [[spindle cell]]s with minimal [[nuclear]] atypia are characteristic findings of Kaposi's sarcoma. Kaposi's sarcoma may be classified according to the clinical setting of the disease into five subtypess: classic Kaposi's sarcoma, African cutaneous Kaposi's sarcoma, African lymphadenopathic Kaposi's sarcoma, iatrogenic Kaposi's sarcoma, and AIDS-associated Kaposi's sarcoma. The epidemiology of Kaposi sarcoma varies depending on its on the specific subtype. Before the [[AIDS]] epidimic, Kaposi's sarcoma was relatively rare. In the early epidemic of [[AIDS]], the incidence of HIV-related Kaposi sarcoma significantly increased; however, [[antiretroviral therapy]] led to a decrease in the incidence of HIV-related Kaposi sarcoma. While endemic African Kaposi sarcoma is more common in African Bantu, classic Kaposi sarcoma is more prevalent in Middle Eastern countries particularly among males and individuals from Jewish descent. The incidence of Kaposi sarcoma is markedly increased in renal transplant patients ranging from 0.5% to 5.3%. Transplant-related Kaposi sarcoma occurs more commonly in individuals who have Jewish, Arabic, Mediterranean, African, or Caribbean ethnicity. In the United States, the age-adjusted [[prevalence]] of Kaposi sarcoma is 8.1 per 100,000 in 2011. The most potent risk factor in the development of Kaposi's sarcoma is an immune deficiency state. There is no established system for the staging of Kaposi's sarcoma among [[immune]] competent patients. However, the staging of [[AIDS]]-associated Kaposi's sarcoma is based on the AIDS Clinical Trials Group (ACTG) staging system. Symptoms of Kaposi's sarcoma include [[fever]], [[abdominal pain]], [[hematochezia]], [[hemoptysis]], and gradual development of red/violaceous [[macule]]s and patches over the nose, legs, and feet. The optimal therapy for Kaposi's sarcoma depends on multiple factors. Management strategies varies depending on the specific variant of Kaposi's sarcoma. Classic Kaposi's sarcoma management may range from no treatment to either [[radiotherapy]], local therapeutic interventions, or surgical excision. Iatrogenic Kaposi's sarcoma management focuses on modifying [[immunosuppressive therapy]] in addition to local therapeutic interventions. Endemic Kaposi's sarcoma is primarily manage by systemic [[chemotherapy]]. However, there is no curative treatment for epidemic Kaposi's sarcoma; the mainstay management for such patients is [[Highly active antiretroviral therapy|HAART therapy]] which aims for the control of Kaposi's sarcoma progression. Local/regional therapy recommended for the management of Kaposi's sarcoma patients may include surgical excision, [[cryotherapy]], and [[sclerotherapy]]. |
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| ==Historical Perspective== | | ==Historical Perspective== |
| Kaposi's sarcoma was first described by Dr. Moritz Kaposi, a Hungarian [[dermatologist]] at the University of Vienna, in the year 1872.<ref name="wiki">Kaposi's sarcoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Kaposi's_sarcoma Accessed on January, 17 2015</ref><ref name="radio"> Kaposi's Sarcoma. Radiopaedia (2015) http://radiopaedia.org/articles/kaposi-sarcoma Accessed on January, 19 2016</ref><ref name="a">{{cite journal |last=Kaposi|first=M |authorlink= |coauthors= |year=1872 |month= |title=Idiopathisches multiples Pigmentsarkom der Haut |journal=Arch. Dermatol. Syph. |volume=4 |issue= |pages=265-273 |id= |url= |accessdate= |quote= }}</ref> | | Kaposi's sarcoma was first described by Dr. Moritz Kaposi, a Hungarian [[dermatologist]] at the University of Vienna, in the year 1872. |
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| ==Classification== | | ==Classification== |
| Kaposi's sarcoma may be classified according to the clinical setting of the disease into five subtypess: classic Kaposi's sarcoma, African cutaneous Kaposi's sarcoma, African lymphadenopathic Kaposi's sarcoma, iatrogenic Kaposi's sarcoma, and AIDS-associated Kaposi's sarcoma.<ref name="wiki">Kaposi's sarcoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Kaposi's_sarcoma Accessed on January, 17 2015</ref> | | Kaposi's sarcoma may be classified according to the clinical setting of the disease into five subtypess: classic Kaposi's sarcoma, African cutaneous Kaposi's sarcoma, African lymphadenopathic Kaposi's sarcoma, iatrogenic Kaposi's sarcoma, and AIDS-associated Kaposi's sarcoma. |
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| ==Pathophysiology== | | ==Pathophysiology== |
| Kaposi's sarcoma arises from [[endothelial cell]]s, which are [[epithelial cell]]s that normally lines the luminal surface of [[blood vessel]]s and [[lymphatic]] vessels.<ref name="pmid23806158">{{cite journal| author=Ruocco E, Ruocco V, Tornesello ML, Gambardella A, Wolf R, Buonaguro FM| title=Kaposi's sarcoma: etiology and pathogenesis, inducing factors, causal associations, and treatments: facts and controversies. | journal=Clin Dermatol | year= 2013 | volume= 31 | issue= 4 | pages= 413-22 | pmid=23806158 | doi=10.1016/j.clindermatol.2013.01.008 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23806158 }} </ref><ref name="patho2"> Kaposi's Sarcoma. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/skintumornonmelanocytickaposisarcoma.html Accessed on January, 19 2015</ref><ref name="wiki">Kaposi's sarcoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Kaposi's_sarcoma Accessed on January, 17 2015</ref> Kaposi's sarcoma is mainly caused by an [[infection]] with Human herpes virus 8 ([[HHV-8]]), which is also known as Kaposi's sarcoma-associated herpes virus (KSHV). The main [[gene]] involved in the pathogensis of Kaposi's sarcoma is ORF73 gene, which encodes the viral latency-associated [[nuclear]] [[antigen]] (LANA-1).<ref name="pmid23698402">{{cite journal| author=Toth Z, Brulois K, Jung JU| title=The chromatin landscape of Kaposi's sarcoma-associated herpesvirus. | journal=Viruses | year= 2013 | volume= 5 | issue= 5 | pages= 1346-73 | pmid=23698402 | doi=10.3390/v5051346 | pmc=PMC3712311 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23698402 }} </ref> Kaposi's sarcoma is commonly associated with acquired immune deficiency syndrome ([[AIDS]]). On gross pathology, reddish, violaceous, or bluish-black [[macule]]s and patches are characteristic findings of Kaposi's sarcoma. On microscopic histopathological analysis the presence of [[spindle cell]]s with minimal nuclear atypia are characteristic findings of Kaposi's sarcoma.<ref name="pmid25375885">{{cite journal| author=Rossetto CC, Pari GS| title=PAN's Labyrinth: Molecular biology of Kaposi's sarcoma-associated herpesvirus (KSHV) PAN RNA, a multifunctional long noncoding RNA. | journal=Viruses | year= 2014 | volume= 6 | issue= 11 | pages= 4212-26 | pmid=25375885 | doi=10.3390/v6114212 | pmc=PMC4246217 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25375885 }} </ref><ref name="pmid23685018">{{cite journal| author=Cancian L, Hansen A, Boshoff C| title=Cellular origin of Kaposi's sarcoma and Kaposi's sarcoma-associated herpesvirus-induced cell reprogramming. | journal=Trends Cell Biol | year= 2013 | volume= 23 | issue= 9 | pages= 421-32 | pmid=23685018 | doi=10.1016/j.tcb.2013.04.001 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23685018 }} </ref><ref name="pmid25068226">{{cite journal| author=Zattra E Coati I, Alaibac M, Piaserico S| title=Kaposi's sarcoma and other rare skin cancers in organ transplant patients. | journal=G Ital Dermatol Venereol | year= 2014 | volume= 149 | issue= 4 | pages= 395-400 | pmid=25068226 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25068226 }} </ref><ref name="radio"> Kaposi's Sarcoma. Radiopaedia (2015) http://radiopaedia.org/articles/kaposi-sarcoma Accessed on January, 19 2016</ref> | | Kaposi's sarcoma arises from [[endothelial cell]]s, which are [[epithelial cell]]s that normally lines the luminal surface of [[blood vessel]]s and [[lymphatic]] vessels. Kaposi's sarcoma is mainly caused by an [[infection]] with Human herpes virus 8 ([[HHV-8]]), which is also known as Kaposi's sarcoma-associated herpes virus (KSHV). The main [[gene]] involved in the pathogensis of Kaposi's sarcoma is ORF73 gene, which encodes the viral latency-associated [[nuclear]] [[antigen]] (LANA-1). Kaposi's sarcoma is commonly associated with acquired immune deficiency syndrome ([[AIDS]]). On gross pathology, reddish, violaceous, or bluish-black [[macule]]s and patches are characteristic findings of Kaposi's sarcoma. On microscopic histopathological analysis the presence of [[spindle cell]]s with minimal nuclear atypia are characteristic findings of Kaposi's sarcoma. |
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| ==Causes== | | ==Causes== |
| Kaposi's sarcoma is caused by an infection with [[HHV-8]].<ref name="pmid23806158">{{cite journal| author=Ruocco E, Ruocco V, Tornesello ML, Gambardella A, Wolf R, Buonaguro FM| title=Kaposi's sarcoma: etiology and pathogenesis, inducing factors, causal associations, and treatments: facts and controversies. | journal=Clin Dermatol | year= 2013 | volume= 31 | issue= 4 | pages= 413-22 | pmid=23806158 | doi=10.1016/j.clindermatol.2013.01.008 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23806158 }} </ref><ref name="patho2"> Kaposi's Sarcoma. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/skintumornonmelanocytickaposisarcoma.html Accessed on January, 19 2015</ref><ref name="wiki">Kaposi's sarcoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Kaposi's_sarcoma Accessed on January, 17 2015</ref> | | Kaposi's sarcoma is caused by an infection with [[HHV-8]]. |
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| ==Differentiating Kaposi's sarcoma from other Diseases== | | ==Differentiating Kaposi's sarcoma from other Diseases== |
| Kaposi's sarcoma must be differentiated from other diseases that cause similar [[cutaneous]], [[pulmonary]], and [[gastrointestinal]] involvement, such as [[bacillary angiomatosis]], [[AIDS]]-related [[lymphoma]], and [[seborrheic keratosis]].<ref name="patho2"> Kaposi's Sarcoma. PathologyOutlines (2015) http://www.pathologyoutlines.com/topic/skintumornonmelanocytickaposisarcoma.html Accessed on January, 19 2015</ref><ref name="radio"> Kaposi's Sarcoma. Radiopaedia (2015) http://radiopaedia.org/articles/kaposi-sarcoma Accessed on January, 19 2016</ref><ref name="patho">Libre Pathology. Kaposi's sarcoma (2015) http://librepathology.org/wiki/index.php/File:Kaposi_sarcoma_low_intermed_mag.jpg Accessed on January, 19 2016</ref> | | Kaposi's sarcoma must be differentiated from other diseases that cause similar [[cutaneous]], [[pulmonary]], and [[gastrointestinal]] involvement, such as [[bacillary angiomatosis]], [[AIDS]]-related [[lymphoma]], and [[seborrheic keratosis]]. |
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| ==Epidemiology and Demographics== | | ==Epidemiology and Demographics== |
| The epidemiology of Kaposi sarcoma varies depending on the specific subtype. Before the [[AIDS]] epidemic, Kaposi's sarcoma was relatively rare. In the early epidemic of [[AIDS]], the incidence of HIV-related Kaposi sarcoma significantly increased;<ref name=cancer.gov>[http://www.cancer.org/cancer/kaposisarcoma/detailedguide/kaposi-sarcoma-what-is-key-statistics What are the key statistics about Kaposi sarcoma? Cancer.org]</ref> however, [[antiretroviral therapy]] led to a decrease in the incidence of HIV-related Kaposi sarcoma.<ref name="pmid12853764">{{cite journal| author=Portsmouth S, Stebbing J, Gill J, Mandalia S, Bower M, Nelson M et al.| title=A comparison of regimens based on non-nucleoside reverse transcriptase inhibitors or protease inhibitors in preventing Kaposi's sarcoma. | journal=AIDS | year= 2003 | volume= 17 | issue= 11 | pages= F17-22 | pmid=12853764 | doi=10.1097/01.aids.0000076309.76477.f1 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12853764 }} </ref><ref name="pmid16849755">{{cite journal| author=Grabar S, Abraham B, Mahamat A, Del Giudice P, Rosenthal E, Costagliola D| title=Differential impact of combination antiretroviral therapy in preventing Kaposi's sarcoma with and without visceral involvement. | journal=J Clin Oncol | year= 2006 | volume= 24 | issue= 21 | pages= 3408-14 | pmid=16849755 | doi=10.1200/JCO.2005.05.4072 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16849755 }} </ref> While endemic African Kaposi sarcoma is more common in African Bantu,<ref name="pmid1765111">{{cite journal| author=Wahman A, Melnick SL, Rhame FS, Potter JD| title=The epidemiology of classic, African, and immunosuppressed Kaposi's sarcoma. | journal=Epidemiol Rev | year= 1991 | volume= 13 | issue= | pages= 178-99 | pmid=1765111 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1765111 }} </ref> classic Kaposi sarcoma is more prevalent in Middle Eastern countries particularly among males and individuals from Jewish descent.<ref name="pmid1765111">{{cite journal| author=Wahman A, Melnick SL, Rhame FS, Potter JD| title=The epidemiology of classic, African, and immunosuppressed Kaposi's sarcoma. | journal=Epidemiol Rev | year= 1991 | volume= 13 | issue= | pages= 178-99 | pmid=1765111 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1765111 }} </ref><ref name="pmid8804445">{{cite journal| author=Franceschi S, Geddes M| title=Epidemiology of classic Kaposi's sarcoma, with special reference to mediterranean population. | journal=Tumori | year= 1995 | volume= 81 | issue= 5 | pages= 308-14 | pmid=8804445 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8804445 }} </ref> The incidence of Kaposi sarcoma is markedly increased in renal transplant patients ranging from 0.5% to 5.3%.<ref name="pmid17456614">{{cite journal| author=Campistol JM, Schena FP| title=Kaposi's sarcoma in renal transplant recipients--the impact of proliferation signal inhibitors. | journal=Nephrol Dial Transplant | year= 2007 | volume= 22 Suppl 1 | issue= | pages= i17-22 | pmid=17456614 | doi=10.1093/ndt/gfm089 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17456614 }} </ref> Transplant-related Kaposi sarcoma occurs more commonly in individuals who have Jewish, Arabic, Mediterranean, African, or Caribbean ethnicity.<ref name="pmid17456614">{{cite journal| author=Campistol JM, Schena FP| title=Kaposi's sarcoma in renal transplant recipients--the impact of proliferation signal inhibitors. | journal=Nephrol Dial Transplant | year= 2007 | volume= 22 Suppl 1 | issue= | pages= i17-22 | pmid=17456614 | doi=10.1093/ndt/gfm089 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17456614 }} </ref> In the United States, the age-adjusted [[prevalence]] of Kaposi sarcoma is 8.1 per 100,000 in 2011.<ref name="SEER">Howlader N, Noone AM, Krapcho M, Garshell J, Miller D, Altekruse SF, Kosary CL, Yu M, Ruhl J, Tatalovich Z,Mariotto A, Lewis DR, Chen HS, Feuer EJ, Cronin KA (eds). SEER Cancer Statistics Review, 1975-2011, National Cancer Institute. Bethesda, MD, http://seer.cancer.gov/csr/1975_2011/, based on November 2013 SEER data submission, posted to the SEER web site, April 2014.</ref> | | The epidemiology of Kaposi sarcoma varies depending on the specific subtype. Before the [[AIDS]] epidemic, Kaposi's sarcoma was relatively rare. In the early epidemic of [[AIDS]], the incidence of HIV-related Kaposi sarcoma significantly increased; however, [[antiretroviral therapy]] led to a decrease in the incidence of HIV-related Kaposi sarcoma. While endemic African Kaposi sarcoma is more common in African Bantu, classic Kaposi sarcoma is more prevalent in Middle Eastern countries particularly among males and individuals from Jewish descent. The incidence of Kaposi sarcoma is markedly increased in renal transplant patients ranging from 0.5% to 5.3%. Transplant-related Kaposi sarcoma occurs more commonly in individuals who have Jewish, Arabic, Mediterranean, African, or Caribbean ethnicity. In the United States, the age-adjusted [[prevalence]] of Kaposi sarcoma is 8.1 per 100,000 in 2011. |
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| ==Risk Factors== | | ==Risk Factors== |
| The most potent risk factor in the development of Kaposi's sarcoma is an [[immune deficiency]] state. Other risk factors include multiple sexual partners, homosexual males, and medical procedures such as blood transfusions and organ transplantation.<ref name="wiki">Kaposi's sarcoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Kaposi's_sarcoma Accessed on January, 17 2015</ref><ref name="cancer"> Sarcoma- Kaposi Guide. Cancer.Net (2015) http://www.cancer.net/cancer-types/sarcoma-kaposi/risk-factors-and-prevention Accessed on January, 20 2015</ref> | | The most potent risk factor in the development of Kaposi's sarcoma is an [[immune deficiency]] state. Other risk factors include multiple sexual partners, homosexual males, and medical procedures such as blood transfusions and organ transplantation. |
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| ==Screening== | | ==Screening== |
| According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for Kaposi's sarcoma.<ref name="US"> Recommendations. U.S. Preventive Service (2015) http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=kaposi+sarcoma Accessed on January, 20 2015</ref> | | According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for Kaposi's sarcoma. |
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| ==Natural History, Complications and Prognosis== | | ==Natural History, Complications and Prognosis== |
| The prognosis of Kaposi's sarcoma depends on its histological type, whether the cancer has spread, and the general health status of the patient. When stratified by age, the 5-year relative survival of patients with Kaposi sarcoma was 68.6% and 87.5% for patients <65 and ≥ 65 years of age respectively.<ref name="SEER">Howlader N, Noone AM, Krapcho M, Garshell J, Miller D, Altekruse SF, Kosary CL, Yu M, Ruhl J, Tatalovich Z,Mariotto A, Lewis DR, Chen HS, Feuer EJ, Cronin KA (eds). SEER Cancer Statistics Review, 1975-2011, National Cancer Institute. Bethesda, MD, http://seer.cancer.gov/csr/1975_2011/, based on November 2013 SEER data submission, posted to the SEER web site, April 2014.</ref> | | The prognosis of Kaposi's sarcoma depends on its histological type, whether the cancer has spread, and the general health status of the patient. When stratified by age, the 5-year relative survival of patients with Kaposi sarcoma was 68.6% and 87.5% for patients <65 and ≥ 65 years of age respectively. |
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| ==Diagnosis== | | ==Diagnosis== |
| ===Staging=== | | ===Staging=== |
| There is no established system for the staging of Kaposi's sarcoma among [[immune]] competent patients. However, the staging of [[AIDS]]-associated Kaposi's sarcoma is based on the AIDS Clinical Trials Group (ACTG) staging system. According to the ACTG staging system, there are 2 groups of AIDS-associated Kaposi's sarcoma based on the [[tumor]] size, patients immune status, and the presence of any systemic illness.<ref name="Nation">Kaposi Sarcoma Treatment–for health professionals. National Cancer Institute (2015) http://www.cancer.gov/types/soft-tissue-sarcoma/hp/kaposi-treatment-pdq#section/_15 Accessed on January, 20 2015</ref><ref name="cancer"> Sarcoma- Kaposi Guide. Cancer.Net (2015) http://www.cancer.net/cancer-types/sarcoma-kaposi/risk-factors-and-prevention Accessed on January, 20 2015</ref><ref name="pmid2671281">{{cite journal| author=Krown SE, Metroka C, Wernz JC| title=Kaposi's sarcoma in the acquired immune deficiency syndrome: a proposal for uniform evaluation, response, and staging criteria. AIDS Clinical Trials Group Oncology Committee. | journal=J Clin Oncol | year= 1989 | volume= 7 | issue= 9 | pages= 1201-7 | pmid=2671281 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2671281 }} </ref><ref name="pmid9294471">{{cite journal| author=Krown SE, Testa MA, Huang J| title=AIDS-related Kaposi's sarcoma: prospective validation of the AIDS Clinical Trials Group staging classification. AIDS Clinical Trials Group Oncology Committee. | journal=J Clin Oncol | year= 1997 | volume= 15 | issue= 9 | pages= 3085-92 | pmid=9294471 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9294471 }} </ref><ref name="america"> How is Kaposi sarcoma staged? American Cancer Association (2015) http://www.cancer.org/cancer/kaposisarcoma/detailedguide/kaposi-sarcoma-staging Accessed on January, 20 2016</ref> | | There is no established system for the staging of Kaposi's sarcoma among [[immune]] competent patients. However, the staging of [[AIDS]]-associated Kaposi's sarcoma is based on the AIDS Clinical Trials Group (ACTG) staging system. According to the ACTG staging system, there are 2 groups of AIDS-associated Kaposi's sarcoma based on the [[tumor]] size, patients immune status, and the presence of any systemic illness. |
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| ===History and Symptoms=== | | ===History and Symptoms=== |
| Symptoms of Kaposi's sarcoma include [[fever]], [[abdominal pain]], [[hematochezia]], [[hemoptysis]], and gradual development of red/violaceous [[macule]]s and patches over the nose, legs, and feet.<ref name="wiki">Kaposi's sarcoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Kaposi's_sarcoma Accessed on January, 17 2015</ref><ref name="Nation">Kaposi Sarcoma Treatment–for health professionals. National Cancer Institute (2015) http://www.cancer.gov/types/soft-tissue-sarcoma/hp/kaposi-treatment-pdq#section/_15 Accessed on January, 20 2015</ref><ref name="cancer"> Sarcoma- Kaposi Guide. Cancer.Net (2015) http://www.cancer.net/cancer-types/sarcoma-kaposi/risk-factors-and-prevention Accessed on January, 20 2015</ref><ref name="pmid21963166">{{cite journal| author=Restrepo CS, Ocazionez D| title=Kaposi's sarcoma: imaging overview. | journal=Semin Ultrasound CT MR | year= 2011 | volume= 32 | issue= 5 | pages= 456-69 | pmid=21963166 | doi=10.1053/j.sult.2011.03.007 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21963166 }} </ref> | | Symptoms of Kaposi's sarcoma include [[fever]], [[abdominal pain]], [[hematochezia]], [[hemoptysis]], and gradual development of red/violaceous [[macule]]s and patches over the nose, legs, and feet. |
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| ===Physical Examination=== | | ===Physical Examination=== |
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| ===Laboratory Findings=== | | ===Laboratory Findings=== |
| Laboratory findings consistent with the diagnosis of Kaposi's sarcoma include decreased [[hemoglobin]] level, positive [[HIV]] tests, and positive [[HHV-8]] [[antigen]] on immunohistochemistry.<ref name="wiki">Kaposi's sarcoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Kaposi's_sarcoma Accessed on January, 17 2015</ref><ref name="cancer"> Sarcoma- Kaposi Guide. Cancer.Net (2015) http://www.cancer.net/cancer-types/sarcoma-kaposi/risk-factors-and-prevention Accessed on January, 20 2015</ref> | | Laboratory findings consistent with the diagnosis of Kaposi's sarcoma include decreased [[hemoglobin]] level, positive [[HIV]] tests, and positive [[HHV-8]] [[antigen]] on immunohistochemistry. |
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| ===Chest X-Ray=== | | ===Chest X-Ray=== |
| Chest X-ray may be helpful in the diagnosis of Kaposi's sarcoma lesions that are located in the [[lung]]s. Findings on chest X-ray suggestive of Kaposi's sarcoma include [[parenchyma|parenchymal]] [[nodule|nodular]] or reticular opacities, [[pleural effusion]], and [[mediastinal]]/[[hilar]] [[lymphadenopathy]].<ref name="radio"> Kaposi's Sarcoma. Radiopaedia (2015) http://radiopaedia.org/articles/kaposi-sarcoma Accessed on January, 19 2016</ref><ref name="pmid21963166">{{cite journal| author=Restrepo CS, Ocazionez D| title=Kaposi's sarcoma: imaging overview. | journal=Semin Ultrasound CT MR | year= 2011 | volume= 32 | issue= 5 | pages= 456-69 | pmid=21963166 | doi=10.1053/j.sult.2011.03.007 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21963166 }} </ref> | | Chest X-ray may be helpful in the diagnosis of Kaposi's sarcoma lesions that are located in the [[lung]]s. Findings on chest X-ray suggestive of Kaposi's sarcoma include [[parenchyma|parenchymal]] [[nodule|nodular]] or reticular opacities, [[pleural effusion]], and [[mediastinal]]/[[hilar]] [[lymphadenopathy]]. |
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| ===CT Scan=== | | ===CT Scan=== |
| CT scan may be helpful in the diagnosis of Kaposi's sarcoma lesions. Findings on CT scan of the chest suggestive of Kaposi's sarcoma include ill-defined (flame-shaped) [[nodular]] lung opacities, interlobular [[septal]] thickening, and hilar [[lymphadenopathy]].<ref name="radio"> Kaposi's Sarcoma. Radiopaedia (2015) http://radiopaedia.org/articles/kaposi-sarcoma Accessed on January, 19 2016</ref> | | CT scan may be helpful in the diagnosis of Kaposi's sarcoma lesions. Findings on CT scan of the chest suggestive of Kaposi's sarcoma include ill-defined (flame-shaped) [[nodular]] lung opacities, interlobular [[septal]] thickening, and hilar [[lymphadenopathy]]. |
| <ref name="pmid21963166">{{cite journal| author=Restrepo CS, Ocazionez D| title=Kaposi's sarcoma: imaging overview. | journal=Semin Ultrasound CT MR | year= 2011 | volume= 32 | issue= 5 | pages= 456-69 | pmid=21963166 | doi=10.1053/j.sult.2011.03.007 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21963166 }} </ref>
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| ===Other Imaging Findings=== | | ===Other Imaging Findings=== |
| [[Scintigraphy]] may be helpful in the diagnosis of Kaposi's sarcoma.<ref name="radio"> Kaposi's Sarcoma. Radiopaedia (2015) http://radiopaedia.org/articles/kaposi-sarcoma Accessed on January, 19 2016</ref><ref name="pmid21963166">{{cite journal| author=Restrepo CS, Ocazionez D| title=Kaposi's sarcoma: imaging overview. | journal=Semin Ultrasound CT MR | year= 2011 | volume= 32 | issue= 5 | pages= 456-69 | pmid=21963166 | doi=10.1053/j.sult.2011.03.007 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21963166 }} </ref> | | [[Scintigraphy]] may be helpful in the diagnosis of Kaposi's sarcoma. |
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| ===Other Diagnostic Studies=== | | ===Other Diagnostic Studies=== |
| Other diagnostic studies for Kaposi's sarcoma include endoscopy, bronchoscopy, and biopsy.<ref name="cancer"> Sarcoma- Kaposi Guide. Cancer.Net (2015) http://www.cancer.net/cancer-types/sarcoma-kaposi/risk-factors-and-prevention Accessed on January, 20 2015</ref> | | Other diagnostic studies for Kaposi's sarcoma include endoscopy, bronchoscopy, and biopsy. |
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| ==Treatment== | | ==Treatment== |
| ===Medical Therapy=== | | ===Medical Therapy=== |
| The optimal therapy for Kaposi's sarcoma depends on multiple factors. Management strategies varies depending on the specific variant of Kaposi's sarcoma. Classic Kaposi's sarcoma management may range from no treatment to either [[radiotherapy]], local therapeutic interventions, or surgical excision. Iatrogenic Kaposi's sarcoma management focuses on modifying [[immunosuppressive therapy]] in addition to local therapeutic interventions. Endemic Kaposi's sarcoma is primarily manage by systemic [[chemotherapy]]. However, there is no curative treatment for epidemic Kaposi's sarcoma; the mainstay management for such patients is [[Highly active antiretroviral therapy|HAART therapy]] which aims for the control of Kaposi's sarcoma progression.<ref name="pmid22677687">{{cite journal| author=Fatahzadeh M| title=Kaposi sarcoma: review and medical management update. | journal=Oral Surg Oral Med Oral Pathol Oral Radiol | year= 2012 | volume= 113 | issue= 1 | pages= 2-16 | pmid=22677687 | doi=10.1016/j.tripleo.2011.05.011 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22677687 }} </ref><ref name="pmid24585567">{{cite journal| author=Masur H, Brooks JT, Benson CA, Holmes KK, Pau AK, Kaplan JE et al.| title=Prevention and treatment of opportunistic infections in HIV-infected adults and adolescents: Updated Guidelines from the Centers for Disease Control and Prevention, National Institutes of Health, and HIV Medicine Association of the Infectious Diseases Society of America. | journal=Clin Infect Dis | year= 2014 | volume= 58 | issue= 9 | pages= 1308-11 | pmid=24585567 | doi=10.1093/cid/ciu094 | pmc=PMC3982842 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24585567 }} </ref> | | The optimal therapy for Kaposi's sarcoma depends on multiple factors. Management strategies varies depending on the specific variant of Kaposi's sarcoma. Classic Kaposi's sarcoma management may range from no treatment to either [[radiotherapy]], local therapeutic interventions, or surgical excision. Iatrogenic Kaposi's sarcoma management focuses on modifying [[immunosuppressive therapy]] in addition to local therapeutic interventions. Endemic Kaposi's sarcoma is primarily manage by systemic [[chemotherapy]]. However, there is no curative treatment for epidemic Kaposi's sarcoma; the mainstay management for such patients is [[Highly active antiretroviral therapy|HAART therapy]] which aims for the control of Kaposi's sarcoma progression. |
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| ===Surgery=== | | ===Surgery=== |
| Local/regional therapy recommended for the management of Kaposi's sarcoma patients may include surgical excision, [[cryotherapy]], and [[sclerotherapy]].<ref name="pmid22677687">{{cite journal| author=Fatahzadeh M| title=Kaposi sarcoma: review and medical management update. | journal=Oral Surg Oral Med Oral Pathol Oral Radiol | year= 2012 | volume= 113 | issue= 1 | pages= 2-16 | pmid=22677687 | doi=10.1016/j.tripleo.2011.05.011 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22677687 }} </ref> | | Local/regional therapy recommended for the management of Kaposi's sarcoma patients may include surgical excision, [[cryotherapy]], and [[sclerotherapy]]. |
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| ==References== | | ==References== |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2]
Overview
Kaposi's sarcoma arises from endothelial cells, which are epithelial cells that normally lines the luminal surface of blood vessels and lymphatic vessels. Kaposi's sarcoma is mainly caused by an infection with Human herpes virus 8 (HHV-8), which is also known as Kaposi's sarcoma-associated herpes virus (KSHV). The main gene involved in the pathogensis of Kaposi's sarcoma is ORF73 gene, which encodes the viral latency-associated nuclear antigen (LANA-1). Kaposi's sarcoma is commonly associated with acquired immune deficiency syndrome (AIDS). On gross pathology, reddish, violaceous, or bluish-black macules and patches are characteristic findings of Kaposi's sarcoma. On microscopic histopathological analysis the presence of spindle cells with minimal nuclear atypia are characteristic findings of Kaposi's sarcoma. Kaposi's sarcoma may be classified according to the clinical setting of the disease into five subtypess: classic Kaposi's sarcoma, African cutaneous Kaposi's sarcoma, African lymphadenopathic Kaposi's sarcoma, iatrogenic Kaposi's sarcoma, and AIDS-associated Kaposi's sarcoma. The epidemiology of Kaposi sarcoma varies depending on its on the specific subtype. Before the AIDS epidimic, Kaposi's sarcoma was relatively rare. In the early epidemic of AIDS, the incidence of HIV-related Kaposi sarcoma significantly increased; however, antiretroviral therapy led to a decrease in the incidence of HIV-related Kaposi sarcoma. While endemic African Kaposi sarcoma is more common in African Bantu, classic Kaposi sarcoma is more prevalent in Middle Eastern countries particularly among males and individuals from Jewish descent. The incidence of Kaposi sarcoma is markedly increased in renal transplant patients ranging from 0.5% to 5.3%. Transplant-related Kaposi sarcoma occurs more commonly in individuals who have Jewish, Arabic, Mediterranean, African, or Caribbean ethnicity. In the United States, the age-adjusted prevalence of Kaposi sarcoma is 8.1 per 100,000 in 2011. The most potent risk factor in the development of Kaposi's sarcoma is an immune deficiency state. There is no established system for the staging of Kaposi's sarcoma among immune competent patients. However, the staging of AIDS-associated Kaposi's sarcoma is based on the AIDS Clinical Trials Group (ACTG) staging system. Symptoms of Kaposi's sarcoma include fever, abdominal pain, hematochezia, hemoptysis, and gradual development of red/violaceous macules and patches over the nose, legs, and feet. The optimal therapy for Kaposi's sarcoma depends on multiple factors. Management strategies varies depending on the specific variant of Kaposi's sarcoma. Classic Kaposi's sarcoma management may range from no treatment to either radiotherapy, local therapeutic interventions, or surgical excision. Iatrogenic Kaposi's sarcoma management focuses on modifying immunosuppressive therapy in addition to local therapeutic interventions. Endemic Kaposi's sarcoma is primarily manage by systemic chemotherapy. However, there is no curative treatment for epidemic Kaposi's sarcoma; the mainstay management for such patients is HAART therapy which aims for the control of Kaposi's sarcoma progression. Local/regional therapy recommended for the management of Kaposi's sarcoma patients may include surgical excision, cryotherapy, and sclerotherapy.
Historical Perspective
Kaposi's sarcoma was first described by Dr. Moritz Kaposi, a Hungarian dermatologist at the University of Vienna, in the year 1872.
Classification
Kaposi's sarcoma may be classified according to the clinical setting of the disease into five subtypess: classic Kaposi's sarcoma, African cutaneous Kaposi's sarcoma, African lymphadenopathic Kaposi's sarcoma, iatrogenic Kaposi's sarcoma, and AIDS-associated Kaposi's sarcoma.
Pathophysiology
Kaposi's sarcoma arises from endothelial cells, which are epithelial cells that normally lines the luminal surface of blood vessels and lymphatic vessels. Kaposi's sarcoma is mainly caused by an infection with Human herpes virus 8 (HHV-8), which is also known as Kaposi's sarcoma-associated herpes virus (KSHV). The main gene involved in the pathogensis of Kaposi's sarcoma is ORF73 gene, which encodes the viral latency-associated nuclear antigen (LANA-1). Kaposi's sarcoma is commonly associated with acquired immune deficiency syndrome (AIDS). On gross pathology, reddish, violaceous, or bluish-black macules and patches are characteristic findings of Kaposi's sarcoma. On microscopic histopathological analysis the presence of spindle cells with minimal nuclear atypia are characteristic findings of Kaposi's sarcoma.
Causes
Kaposi's sarcoma is caused by an infection with HHV-8.
Differentiating Kaposi's sarcoma from other Diseases
Kaposi's sarcoma must be differentiated from other diseases that cause similar cutaneous, pulmonary, and gastrointestinal involvement, such as bacillary angiomatosis, AIDS-related lymphoma, and seborrheic keratosis.
Epidemiology and Demographics
The epidemiology of Kaposi sarcoma varies depending on the specific subtype. Before the AIDS epidemic, Kaposi's sarcoma was relatively rare. In the early epidemic of AIDS, the incidence of HIV-related Kaposi sarcoma significantly increased; however, antiretroviral therapy led to a decrease in the incidence of HIV-related Kaposi sarcoma. While endemic African Kaposi sarcoma is more common in African Bantu, classic Kaposi sarcoma is more prevalent in Middle Eastern countries particularly among males and individuals from Jewish descent. The incidence of Kaposi sarcoma is markedly increased in renal transplant patients ranging from 0.5% to 5.3%. Transplant-related Kaposi sarcoma occurs more commonly in individuals who have Jewish, Arabic, Mediterranean, African, or Caribbean ethnicity. In the United States, the age-adjusted prevalence of Kaposi sarcoma is 8.1 per 100,000 in 2011.
Risk Factors
The most potent risk factor in the development of Kaposi's sarcoma is an immune deficiency state. Other risk factors include multiple sexual partners, homosexual males, and medical procedures such as blood transfusions and organ transplantation.
Screening
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for Kaposi's sarcoma.
Natural History, Complications and Prognosis
The prognosis of Kaposi's sarcoma depends on its histological type, whether the cancer has spread, and the general health status of the patient. When stratified by age, the 5-year relative survival of patients with Kaposi sarcoma was 68.6% and 87.5% for patients <65 and ≥ 65 years of age respectively.
Diagnosis
Staging
There is no established system for the staging of Kaposi's sarcoma among immune competent patients. However, the staging of AIDS-associated Kaposi's sarcoma is based on the AIDS Clinical Trials Group (ACTG) staging system. According to the ACTG staging system, there are 2 groups of AIDS-associated Kaposi's sarcoma based on the tumor size, patients immune status, and the presence of any systemic illness.
History and Symptoms
Symptoms of Kaposi's sarcoma include fever, abdominal pain, hematochezia, hemoptysis, and gradual development of red/violaceous macules and patches over the nose, legs, and feet.
Physical Examination
Kaposi's sarcoma lesions are nodules or patches that may be purple, red, blue, or black in color and are usually located on the nose, legs, and feet.
Laboratory Findings
Laboratory findings consistent with the diagnosis of Kaposi's sarcoma include decreased hemoglobin level, positive HIV tests, and positive HHV-8 antigen on immunohistochemistry.
Chest X-Ray
Chest X-ray may be helpful in the diagnosis of Kaposi's sarcoma lesions that are located in the lungs. Findings on chest X-ray suggestive of Kaposi's sarcoma include parenchymal nodular or reticular opacities, pleural effusion, and mediastinal/hilar lymphadenopathy.
CT Scan
CT scan may be helpful in the diagnosis of Kaposi's sarcoma lesions. Findings on CT scan of the chest suggestive of Kaposi's sarcoma include ill-defined (flame-shaped) nodular lung opacities, interlobular septal thickening, and hilar lymphadenopathy.
Other Imaging Findings
Scintigraphy may be helpful in the diagnosis of Kaposi's sarcoma.
Other Diagnostic Studies
Other diagnostic studies for Kaposi's sarcoma include endoscopy, bronchoscopy, and biopsy.
Treatment
Medical Therapy
The optimal therapy for Kaposi's sarcoma depends on multiple factors. Management strategies varies depending on the specific variant of Kaposi's sarcoma. Classic Kaposi's sarcoma management may range from no treatment to either radiotherapy, local therapeutic interventions, or surgical excision. Iatrogenic Kaposi's sarcoma management focuses on modifying immunosuppressive therapy in addition to local therapeutic interventions. Endemic Kaposi's sarcoma is primarily manage by systemic chemotherapy. However, there is no curative treatment for epidemic Kaposi's sarcoma; the mainstay management for such patients is HAART therapy which aims for the control of Kaposi's sarcoma progression.
Surgery
Local/regional therapy recommended for the management of Kaposi's sarcoma patients may include surgical excision, cryotherapy, and sclerotherapy.
References
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