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| '''For patient information click [[{{PAGENAME}} (patient information)|here]]'''. | | __NOTOC__ |
| | '''For patient information click [[{{PAGENAME}} (patient information)|here]]'''. |
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| {{DiseaseDisorder infobox | | | {{About1|Kaposi's sarcoma-associated herpesvirus}} |
| Name = Kaposi's sarcoma |
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| ICD10 = {{ICD10|C|46||c|45}} |
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| ICD9 = {{ICD9|176}} |
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| ICDO = {{ICDO|9140|3}} |
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| Image = Kaposi’s sarcoma intraoral AIDS 072 lores.jpg |
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| Caption = <center>Intraoral Kaposi’s sarcoma lesion with an overlying [[candidiasis]] infection.</center>|
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| OMIM = 148000 |
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| MedlinePlus = 000661 |
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| DiseasesDB = 7105 |
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| }} | |
| {{Kaposi's sarcoma}} | | {{Kaposi's sarcoma}} |
| {{CMG}} | | {{CMG}}; {{AE}} {{HL}} {{KS}} {{Rim}} {{ADS}} {{Hudakarman}} |
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| | {{SK}} Kaposi sarcoma; Kaposi sarcomas; Kaposi; KS; Multiple hemorrhagic sarcoma; Non-AIDS associated Kaposi sarcoma; AIDS associated Kaposi sarcoma, KHSV |
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| {{Editor Help}}
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| ==[[Kaposi's sarcoma overview|Overview]]== | | ==[[Kaposi's sarcoma overview|Overview]]== |
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| ==[[Kaposi's sarcoma historical perspective|Historical Perspective]]== | | ==[[Kaposi's sarcoma historical perspective|Historical Perspective]]== |
| | | ==[[Kaposi's sarcoma classification|Classification]]== |
| ==[[Kaposi's sarcoma pathophysiology|Pathophysiology]]== | | ==[[Kaposi's sarcoma pathophysiology|Pathophysiology]]== |
| | | ==[[Kaposi's sarcoma differential diagnosis|Differentiating Kaposi's sarcoma from other Diseases]]== |
| ==[[Kaposi's sarcoma epidemiology and demographics|Epidemiology & Demographics]]== | | ==[[Kaposi's sarcoma epidemiology and demographics|Epidemiology and Demographics]]== |
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| ==[[Kaposi's sarcoma risk factors|Risk Factors]]== | | ==[[Kaposi's sarcoma risk factors|Risk Factors]]== |
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| ==[[Kaposi's sarcoma screening|Screening]]== | | ==[[Kaposi's sarcoma screening|Screening]]== |
| | ==[[Kaposi's sarcoma natural history|Natural History, Complications and Prognosis]]== |
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| ==[[Kaposi's sarcoma causes|Causes]]== | | ==Diagnosis== |
| | | [[Kaposi's sarcoma diagnostic study of choice|Diagnostic Study of Choice]] | [[Kaposi's sarcoma staging|Staging]] | [[Kaposi's sarcoma history and symptoms|History and Symptoms]] | [[Kaposi's sarcoma physical examination|Physical Examination]] | [[Kaposi's sarcoma laboratory findings|Laboratory Findings]] | [[Kaposi's sarcoma electrocardiogram|Electrocardiogram]] | [[Kaposi's sarcoma x ray|Chest X Ray]] | [[Kaposi's sarcoma echocardiograph and ultrasound|Echocardiograph and Ultrasound]] | [[Kaposi's sarcoma CT|CT]] | [[Kaposi's sarcoma mri|MRI]] | [[Kaposi's sarcoma other imaging findings|Other Imaging Findings]] | [[Kaposi's sarcoma other diagnostic studies|Other Diagnostic Studies]] |
| ==[[Kaposi's sarcoma differential diagnosis|Differentiating Kaposi's Sarcoma]]==
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| ==[[Kaposi's sarcoma natural history|Complications & Prognosis]]== | | ==Treatment== |
| | [[Kaposi's sarcoma medical therapy|Medical Therapy]] | [[Kaposi's sarcoma surgery|Surgery]] | [[Kaposi's sarcoma primary prevention|Primary Prevention]] | [[Kaposi's sarcoma secondary prevention|Secondary Prevention]] | [[Kaposi's sarcoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Kaposi's sarcoma future or investigational therapies|Future or Investigational Therapies]] |
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| ==Clinical features== | | ==Case Studies== |
| KS [[lesion]]s are nodules or blotches that may be red, purple, brown, or black, and are usually '''papular''' (ie palpable or raised).
| | [[Kaposi's sarcoma case study one|Case #1]] |
| [[Image:Kaposi's Sarcoma.jpg|thumb|left|Papular cutaneous Kaposi's Sarcoma]]
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| They are typically found on the skin, but spread elsewhere is common, especially the mouth, [[gastrointestinal tract]] and [[respiratory tract]]. Growth can range from very slow to explosively fast, and be associated with significant [[mortality]] and [[morbidity]].<ref>{{cite journal |last=Dezube |first=BJ |authorlink= |coauthors= |year=1996 |month=Oct |title=Clinical presentation and natural history of AIDS--related Kaposi's sarcoma |journal=Hematol Oncol Clin North Am |volume=10 |issue=5 |pages=1023-9 |id= |url=http://www.ncbi.nlm.nih.gov/sites/entrez?cmd=Retrieve&db=PubMed&list_uids=8880194&dopt=Citation |accessdate= |quote= }}</ref>
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| '''Skin'''
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| The skin lesions most commonly affect the lower limbs, face, mouth and [[genitalia]]. The lesions are usually as described above, but may occasionally be [[plaque]] like (often on the soles of the feet) or even involved in skin breakdown with resulting '''fungating''' lesions.
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| Associated swelling may be from either local [[inflammation]] or [[lymphoedema]] (obstruction of local [[lymphatic vessels]] by the lesion). Skin lesions may be quite disfiguring for the sufferer, and a cause of much psychosocial pathology.
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| '''Mouth'''
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| Is involved in about 30%, and is the initial site in 15% of AIDS related KS. In the mouth, the [[hard palate]] is most frequently affected, followed by the [[gums]] <ref>{{cite journal |last=Nichols |first=CM |authorlink= |coauthors= |year=1993 |month=Nov |title=Treating Kaposi's lesions in the HIV-infected patient. |journal=J Am Dent Assoc |volume=124 |issue=11 |pages=78-84 |id= |url=http://jada.ada.org/cgi/content/abstract/124/11/78 |accessdate= 2007-06-11 |quote= }}</ref>. Lesions in the mouth may be easily damaged by chewing and bleed or suffer secondary infection, and even interfere with eating or speaking.
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| '''Gastrointestinal tract'''
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| Involvement can be common in those with transplant related or AIDS related KS, and it may occur in the absence of skin involvement. The gastrointestinal lesions may be silent or cause weight loss, pain, nausea/vomiting, [[diarrhea]], bleeding (either vomiting blood or passing it with bowel motions), [[malabsorption]], or [[intestinal obstruction]].<ref>{{cite journal |last=Danzig |first=JB |authorlink= |coauthors= |year=1991 |month=Jun |title=Gastrointestinal malignancy in patients with AIDS |journal=Am J Gastroenterol |volume=86 |issue=6 |pages=715-8 |id= |url=http://www.ncbi.nlm.nih.gov/sites/entrez?cmd=Retrieve&db=PubMed&list_uids=2038993&dopt=Citation |accessdate= 2007-06-11 |quote= }}</ref>
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| '''Respiratory tract'''
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| Involvement of the airway can present with shortness of breath, [[fever]], [[cough]], [[hemoptysis]] (coughing up blood), or chest pain, or as an incidental finding on [[chest x-ray]].<ref>{{cite journal |last=Garay |first=SM |authorlink= |coauthors= |year=1987 |month=Jan |title=Pulmonary manifestations of Kaposi's sarcoma |journal=Chest |volume=91 |issue=1 |pages=39-43 |id= |url=http://www.chestjournal.org/cgi/reprint/91/1/39 |accessdate= 2007-06-11 |quote= }}</ref> The diagnosis is usually confirmed by [[bronchoscopy]] when the lesions are directly seen, and often biopsied.
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| ==Pathophysiology and diagnosis==
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| Despite its name, it is generally not considered a true [[sarcoma]], which is a tumor arising from [[mesenchyme|mesenchymal tissue]]. KS actually arises as a cancer of [[lymphatic]] [[endothelium]] and forms vascular channels that fill with blood cells, giving the tumor its characteristic bruise-like appearance.
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| KS lesions contain tumor [[cell (biology)|cells]] with a characteristic abnormal elongated shape, called ''spindle cells''. The tumor is highly [[Blood vessel|vascular]], containing abnormally dense and irregular blood vessels, which leak red blood cells into the surrounding tissue and give the tumor its dark color. [[Inflammation]] around the tumor may produce swelling and pain.
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| Although KS may be suspected from the appearance of lesions and the patient's risk factors, a definite diagnosis can only be made by [[biopsy]] and microscopic examination, which will show the presence of spindle cells. Detection of the viral protein LANA in tumor cells confirms the diagnosis.
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| ==Treatment and prevention==
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| Kaposi's sarcoma is not curable, in the usual sense of the word, but it can often be effectively palliated for many years and this is the aim of treatment. In KS associated with [[immunodeficiency]] or immunosuppression, treating the cause of the immune system dysfunction can slow or stop the progression of KS. In 40% or more of patients with AIDS-associated Kaposi's sarcoma, the Kaposi lesions will shrink upon first starting highly active antiretroviral therapy ([[HAART]]). However, in a certain percentage of such patients, Kaposi's sarcoma may again grow after a number of years on HAART, especially if HIV is not completely suppressed. Patients with a few local lesions can often be treated with local measures such as radiation therapy or cryotherapy. Surgery is generally not recommended as Kaposi's sarcoma can appear in wound edges. More widespread disease, or disease affecting internal organs, is generally treated with systemic therapy with [[interferon]] alpha, liposomal anthracyclines (such as Doxil) or [[paclitaxel]].
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| With the decrease in the death rate among AIDS patients receiving new treatments in the 1990s, the incidence and severity of epidemic KS also decreased. However, the number of patients living with AIDS is increasing substantially in the United States, and it is possible that the number of patients with AIDS-associated Kaposi's sarcoma will again rise as these patients live longer with HIV infection.
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| Blood tests to detect antibodies against KSHV have been developed and can be used to determine if a patient is at risk for transmitting infection to his or her sexual partner, or if an organ is infected prior to transplantation.
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| ==History and theories==
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| The disease is named after [[Moritz Kaposi]] (1837–1902), a Hungarian [[dermatologist]] who first described the symptoms in 1872. Research over the next century suggested that KS, like some other forms of cancer, might be caused by a virus or genetic factors, but no definite cause was found.
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| With the rise of the AIDS epidemic, KS, as initially one of the most common AIDS symptoms, was researched more intensively in hopes that it might reveal the cause of AIDS.
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| In 1994, [[Yuan Chang]], [[Patrick S. Moore]], and Ethel Cesarman at Columbia University in New York isolated genetic pieces of a virus from a KS lesion. They used [[representational difference analysis]] (a method to subtract out all of the human DNA from a sample) to isolate the viral genes. They then used these small DNA fragments as starting points to sequence the rest of the viral genome in 1996. This, the eighth human herpesvirus (HHV-8)—now known as Kaposi's sarcoma-associated herpesvirus (KSHV)—has since been found in all KS lesions tested, and is considered the cause of the disease. KSHV is a unique human tumor virus that has incorporated cellular genes that cause tumors into its genome ("molecular piracy"); the stolen cellular genes may help the virus escape from the immune system, but in doing so it also causes cells to proliferate. It is related to [[Epstein-Barr virus]], a very common herpesvirus that can also cause human cancers.
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| KSHV infection does not always lead to KS; it is still unclear what other factors may be required, such as pre-existing immune system damage, or a specific interaction with HIV or other viruses. However, research in Africa has shown that even in the absence of HIV/AIDS, KS is more common in men than women although KSHV infection is equal between both sexes. This suggests that [[sex hormone]]s may either protect from or predispose to KS in persons infected with the virus.
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| ==KS awareness==
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| In AIDS patients, Kaposi's sarcoma is considered an [[opportunistic infection]], ''i.e.,'' a disease that is able to gain a foothold in the body because the immune system has been weakened. With the rise of AIDS in Africa, where KSHV is widespread, KS has become the most frequently reported cancer in some countries, such as Zimbabwe.
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| Nigerian bandleader Fela Kuti succumbed to the disease in 1997.
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| Because of their highly visible nature, external lesions are sometimes the presenting symptom of AIDS. Kaposi's sarcoma entered the awareness of the general public with the release of the film ''Philadelphia'', in which the main character was fired after his employers found out he was HIV-positive due to visible lesions. Unfortunately, by the time KS lesions appear, it is likely that the immune system has already been severely weakened.
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| ==References==
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| {{reflist|2}}
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| {{Soft tissue tumors and sarcomas}} | | {{Soft tissue tumors and sarcomas}} |
| {{SIB}}
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| [[cs:Kaposiho sarkom]] | | [[cs:Kaposiho sarkom]] |
| [[de:Kaposi-Sarkom]] | | [[de:Kaposi-Sarkom]] |
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| [[Category:Pulmonology]] | | [[Category:Pulmonology]] |
| [[Category:Gastroenterology]] | | [[Category:Gastroenterology]] |
| [[Category:Infectious disease]] | | [[Category:Up-To-Date]] |
| [[Category:Mature chapter]] | | [[Category:Oncology]] |
| | [[Category:Medicine]] |
| | [[Category:Vascular medicine]] |
| | [[Category:Surgery]] |