Endemic Kaposi sarcoma: Difference between revisions

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'''Other Names: '''
==Endemic Kaposi Sarcoma==
African Kaposi sarcoma; African/endemic Kaposi sarcoma
'''Kaposi's sarcoma''' ('''KS''') is a type of [[cancer]] that can form masses in the [[skin]], [[lymph nodes]], or other [[organs]]. The skin lesions are usually [[purple]] in color.
They can occur singularly, in a limited area, or be widespread.
Kaposi’s sarcoma (KS) is the most common tumor in HIV-infected individuals in Africa
KS is a [[vascular tumor]] which arises in multifocal sites. The skin is most commonly involved,
though virtually any organ, except perhaps the brain can be involved.
[[File:Kaposis Sarcoma Lesions.jpg|thumb|An example of Kaposi's sarcoma]]
[[Image:Kaposi’s sarcoma intraoral AIDS 072 lores.jpg|thumb|right|An [[HIV]]-positive person presenting with a Kaposi's sarcoma lesion with an overlying [[candidiasis]] infection in their mouth]]


<youtube>
'''Endemic Kaposi Sarcoma''' is a form of [[Kaposi Sarcoma]] that occurs predominantly in certain regions of [[Africa]]. It is one of the four main types of Kaposi Sarcoma, alongside [[epidemic Kaposi Sarcoma]], [[iatrogenic Kaposi Sarcoma]], and [[classic Kaposi Sarcoma]].
title='''{{PAGENAME}}'''
movie_url=http://www.youtube.com/v/gehvdaJUoZE
&rel=1
embed_source_url=http://www.youtube.com/v/gehvdaJUoZE
&rel=1
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== '''Risk factors''' ==
==Epidemiology==
Kaposi's sarcoma remains the most common cancer in Sub-Saharan Africa and the
Endemic Kaposi Sarcoma is most commonly found in [[sub-Saharan Africa]], particularly in countries such as [[Uganda]], [[Zambia]], and [[Malawi]]. It affects both children and adults, with a higher prevalence in males. The disease is more common in areas where [[Human Herpesvirus 8]] (HHV-8), also known as [[Kaposi Sarcoma-associated Herpesvirus]] (KSHV), is endemic.
second most common cancer in HIV-infected patients worldwide. Since the introduction of
[[highly active antiretroviral therapy]] ([[HAART]]), there has been a decline in its incidence.
However, Kaposi's sarcoma continues to be diagnosed in HIV-infected patients.
Risk factors include [[immunosuppression|poor immune function]], either as a result of disease or specific medications, and [[chronic lymphedema]].


==Cause==
==Etiology==
The primary cause of Endemic Kaposi Sarcoma is infection with [[Human Herpesvirus 8]] (HHV-8). The virus is transmitted through [[saliva]], and its prevalence is high in regions where Endemic Kaposi Sarcoma is common. The exact mechanism by which HHV-8 leads to the development of Kaposi Sarcoma is not fully understood, but it is believed to involve the virus's ability to induce [[angiogenesis]] and [[inflammation]].


[[Kaposi's sarcoma-associated herpesvirus]] (KSHV), also called [[HHV-8]], is present in almost 100% of Kaposi sarcoma lesions, whether HIV-related, classic, endemic, or [[iatrogenic]].
==Clinical Presentation==
Endemic Kaposi Sarcoma presents with [[cutaneous]] lesions that are typically [[nodular]] or [[plaque-like]]. These lesions can vary in color from pink to purple and are often found on the [[extremities]], [[face]], and [[oral cavity]]. In some cases, the disease can also affect internal organs, leading to [[visceral Kaposi Sarcoma]].


== '''Classifications''' ==
===Pediatric Endemic Kaposi Sarcoma===
Kasposi’s sarcoma has four classifications, based on varying clinical characteristics and risk
In children, Endemic Kaposi Sarcoma can present with more aggressive features, including [[lymphadenopathy]] and [[visceral involvement]]. Pediatric cases often have a poorer prognosis compared to adults.
factors
* '''Classic Kasposi’s sarcoma''' affects elderly, [[immunocompetent]] individuals of
Mediterranean or Eastern European descent. It is a slow-progressing and relatively benign form
of the cancer.
* '''Endemic or African Kaposi’s sarcoma''' is most common in Central and Eastern
Africa and primarily affects adults
* '''Iatrogenic Kaposi’s sarcoma '''is found in populations with compromised immune systems, primarily in patients who have received organ transplants.
* '''AIDs-Kaposi’s sarcoma''' develops in populations infected with HIV-AIDS. In Western countries,
AIDS-KS is most commonly found in HIV-infected men who have sex with men.


==Signs and symptoms==
==Diagnosis==
KS [[lesion]]s are nodules or blotches that may be red, purple, brown, or black, and are usually [[papule|papular]].
The diagnosis of Endemic Kaposi Sarcoma is primarily clinical, supported by [[histopathological]] examination of biopsy specimens. Histology typically shows [[spindle cell proliferation]], [[vascular slits]], and [[extravasated red blood cells]]. [[Immunohistochemistry]] can be used to detect HHV-8 in tissue samples.


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 is associated with significant [[Mortality rate|mortality]] and [[morbidity]].
==Treatment==
Treatment options for Endemic Kaposi Sarcoma include [[antiretroviral therapy]] (ART) in cases associated with [[HIV/AIDS]], [[chemotherapy]], and [[radiation therapy]]. The choice of treatment depends on the extent of the disease and the patient's overall health status.


The lesions are painless, but become cosmetically disfiguring or interruptive to organs.
==Prognosis==
The prognosis of Endemic Kaposi Sarcoma varies depending on the stage at diagnosis and the presence of [[HIV]] co-infection. Early-stage disease confined to the skin has a better prognosis, while advanced disease with visceral involvement has a poorer outcome.


== '''Diagnostics:''' ==
==Prevention==
First and foremost is the clinical picture of [[erythematous violaceous cutaneous lesions]] that canbe macular, patch, plaque, nodular or exophytic. The lesions can be solitary, localized or
Preventive measures focus on reducing the transmission of HHV-8, particularly in endemic regions. Public health strategies include promoting [[safe practices]] to reduce saliva exchange and improving [[HIV]] prevention and treatment programs.
disseminated. This in the background of HIV/AIDS should alert the physician to the diagnosis of
KS. The presence of local/regional [[lymphoedema]] almost gives away the diagnosis. However
tissue confirmation is mandatory before instituting any form of therapy.


Local punch [[biopsy]] or rarely, excision biopsy are all that are required for a skin biopsy.
==See Also==
* [[Kaposi Sarcoma]]
* [[Human Herpesvirus 8]]
* [[HIV/AIDS]]


[[Lymphnode]] excision can also be done in predominantly nodal lesions. [[Endoscopic biopsies]] may berequired for lesions presenting solely in visceral lumens. Tissues should be subjected to
{{Kaposi Sarcoma}}
pathologic examination by an experienced histopathologist.
{{Infectious diseases}}


== '''Treatment''' ==
[[Category:Oncology]]
It has been noted that [[highly active antiretroviral therapy]] (HAART) alone
[[Category:Infectious diseases]]
improves the outcome of HIV associated KS
[[Category:Dermatology]]
 
[[Category:Viral diseases]]
Patients with aggressive forms of KS are commonly treated with [[paclitaxel]] or [[doxorubicin]](orliposomal doxorubicin), [[bleomycin]] and [[vinblastine]] or [[vincristine]] (ABV). The ABV regimen hasbeen shown to give better response rates than BV (Bleomycin, vinblastine / vincristine) alone. This regimen was however not very popular because of toxicity [[Gemcitabine
[[Category:Medical conditions related to HIV/AIDS]]
monotherapy]] has been suggested as an alternative option in patients previously treated with ABV
 
[[Paclitaxel]], with response rates ranging from 59%-71% when given without HAART (12,13), is
considered the most attractive agent since it is effective and tolerable over long-term
administration especially when combined with  growth factors (13,14). Paclitaxel for this reason
should be added to the essential medicines list.
 
[[Alpha interferon]] and [[radiotherapy]] have also been used in the management of AIDS-associated
KS. Their use has been limited by the toxicity profile. [[Rapamycin]] was noted to be safe in HIV
infected individuals with KS and can in some cases induce tumor regression
 
The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.
* '''[[Pazopanib]]''' (Brand name: Votrient)Advanced soft tissue sarcoma (STS) who have received prior chemotherapy.
 
== '''Additional images''' ==
<gallery>
File:Micrograph of promontory sign of kaposi's sarcoma.jpg|Micrograph of promontory sign in Kaposi's sarcoma in patch stage. Dilated irregular vascular channels surround a pre-existing vessel.<ref name=Soyer2011/>
File:Micrograph of plaque stage of Kaposi's sarcoma.jpg|Micrograph of plaque stage, with bizarre vessels dissecting the upper dermis. There is erythrocyte extravasation and hemosiderin pigmentation.<ref name=Soyer2011/>
File:Micrograph of tumor stage of Kaposi's sarcoma.jpg|Micrograph of tumor stage. Well-circumscribed spindle-cell tumor. Erythrocytes lie within poorly defined slit-like vascular spaces.<ref name=Soyer2011/>
</gallery>
{{Diseases of the skin and appendages by morphology}}
{{Vascular tumors}}
{{Viral cutaneous conditions}}
 
{{DEFAULTSORT:Kaposi sarcoma}}
[[Category:HIV/AIDS]]
[[Category:Virus-related cutaneous conditions]]
[[Category:Connective and soft tissue neoplasms]]
[[Category:Vascular neoplasia]]
[[Category:Rare cancers]]
[[Category:RTT]]
{{rarediseases}}

Latest revision as of 12:37, 31 December 2024

Endemic Kaposi Sarcoma[edit]

Endemic Kaposi Sarcoma is a form of Kaposi Sarcoma that occurs predominantly in certain regions of Africa. It is one of the four main types of Kaposi Sarcoma, alongside epidemic Kaposi Sarcoma, iatrogenic Kaposi Sarcoma, and classic Kaposi Sarcoma.

Epidemiology[edit]

Endemic Kaposi Sarcoma is most commonly found in sub-Saharan Africa, particularly in countries such as Uganda, Zambia, and Malawi. It affects both children and adults, with a higher prevalence in males. The disease is more common in areas where Human Herpesvirus 8 (HHV-8), also known as Kaposi Sarcoma-associated Herpesvirus (KSHV), is endemic.

Etiology[edit]

The primary cause of Endemic Kaposi Sarcoma is infection with Human Herpesvirus 8 (HHV-8). The virus is transmitted through saliva, and its prevalence is high in regions where Endemic Kaposi Sarcoma is common. The exact mechanism by which HHV-8 leads to the development of Kaposi Sarcoma is not fully understood, but it is believed to involve the virus's ability to induce angiogenesis and inflammation.

Clinical Presentation[edit]

Endemic Kaposi Sarcoma presents with cutaneous lesions that are typically nodular or plaque-like. These lesions can vary in color from pink to purple and are often found on the extremities, face, and oral cavity. In some cases, the disease can also affect internal organs, leading to visceral Kaposi Sarcoma.

Pediatric Endemic Kaposi Sarcoma[edit]

In children, Endemic Kaposi Sarcoma can present with more aggressive features, including lymphadenopathy and visceral involvement. Pediatric cases often have a poorer prognosis compared to adults.

Diagnosis[edit]

The diagnosis of Endemic Kaposi Sarcoma is primarily clinical, supported by histopathological examination of biopsy specimens. Histology typically shows spindle cell proliferation, vascular slits, and extravasated red blood cells. Immunohistochemistry can be used to detect HHV-8 in tissue samples.

Treatment[edit]

Treatment options for Endemic Kaposi Sarcoma include antiretroviral therapy (ART) in cases associated with HIV/AIDS, chemotherapy, and radiation therapy. The choice of treatment depends on the extent of the disease and the patient's overall health status.

Prognosis[edit]

The prognosis of Endemic Kaposi Sarcoma varies depending on the stage at diagnosis and the presence of HIV co-infection. Early-stage disease confined to the skin has a better prognosis, while advanced disease with visceral involvement has a poorer outcome.

Prevention[edit]

Preventive measures focus on reducing the transmission of HHV-8, particularly in endemic regions. Public health strategies include promoting safe practices to reduce saliva exchange and improving HIV prevention and treatment programs.

See Also[edit]

Template:Kaposi Sarcoma



Infectious diseases
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