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African Endemic Kaposi Sarcoma

Last updated April 28, 2018

Approved by: Krish Tangella MD, MBA, FCAP

African Endemic Kaposi Sarcoma is observed in people belonging to the sub-Saharan regions of Africa.

What are the other Names for this Condition? (Also known as/Synonyms)

  • African Cutaneous KS
  • African Lymphadenopathic KS
  • Equatorial Africa Kaposi Sarcoma

What is African Endemic Kaposi Sarcoma? (Definition/Background Information)

  • Kaposi Sarcoma (KS) is a malignant tumor that is associated with human herpes viral infection (called HHV8 infection)
  • It manifests as a skin condition with lesions and purple red patches; mainly on the legs, hands, and face. But, it can also affect the lymph nodes, vital organs, and mucous membranes lining the respiratory and digestive system leading to life-threatening situations
  • KS is often linked to HIV-infected individuals. Nevertheless, any individual with a weak immune system, have a risk of being infected by the condition
  • There are 4 types of Kaposi Sarcoma and these include:
    • Classic Indolent KS
    • African Endemic KS
    • Iatrogenic KS
    • AIDS-Related KS
  • African Endemic Kaposi Sarcoma is a form of Kaposi Sarcoma that is unusually aggressive and typically found in individuals of sub-Saharan African regions
  • There are 2 types of African Endemic KS:
    • African Lymphadenopathic Kaposi Sarcoma: It is a very aggressive form that affects very young children
    • African Cutaneous Kaposi Sarcoma: This form of African KS affects young and middle-aged adults
  • The lymphadenopathic form of KS chiefly affects the skin and lymph nodes, while the cutaneous form of KS affects the skin. The African Endemic KS is not related to HIV infection

Who gets African Endemic Kaposi Sarcoma? (Age and Sex Distribution)

  • African Endemic Kaposi Sarcoma is observed in children and adults belonging to the sub-Saharan regions of Africa
  • African Lymphadenopathic KS is observed in young children below 10 years of age
  • African Cutaneous KS affects adults in the 20-50 years age group

What are the Risk Factors for African Endemic Kaposi Sarcoma? (Predisposing Factors)

Risk factors for African Endemic Kaposi Sarcoma include the following:

  • Individuals belonging to sub-Saharan Africa
  • Chronic malaria
  • Chronic parasitic infections
  • Malnutrition

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of African Endemic Kaposi Sarcoma? (Etiology)

  • In general, Kaposi Sarcoma is caused by a complex interaction of the human herpes virus (HHV8), certain genetic factors, a weak immune system, and disease-conducive environmental conditions
  • Medical research has not established the exact mechanism of African Endemic Kaposi Sarcoma formation, even though chromosomal anomalies have been observed in the tumor
  • But the condition is not associated with HIV infection or AIDS

What are the Signs and Symptoms of African Endemic Kaposi Sarcoma?

The signs and symptoms of African Endemic Kaposi Sarcoma, which is a particularly severe and aggressive form of KS, include:

  • The presence of lesions that may be single or multiple in numbers all over the body
  • There are 4 types of Kaposi Sarcoma and these include:
    • In the African Lymphadenopathic form of KS, both skin and lymph nodes are involved
    • In the African Cutaneous form of KS, reddish brown masses are seen on the legs
  • If the skin is affected, then KS may be manifested as a mass on the skin, which may bleed and ulcerate
  • If the mouth or oral cavity is affected, then there may be difficulty in chewing, swallowing, and speaking
  • Vomiting, diarrhea, and weight loss may occur if the gastrointestinal tract is involved
  • Breathing difficulty, cough, blood in sputum, and fever may be an indication that the lungs are infected
  • In rare cases, the internal organs may be affected

How is African Endemic Kaposi Sarcoma Diagnosed?

Diagnosis of African Endemic Kaposi Sarcoma may be established through:

  • Complete physical examination and thorough evaluation of individual’s medical history
  • Blood tests including complete blood count (CBC) and tests to detect antibodies against the virus
  • CT scan of the affected regions
  • Bronchoscopy, if lung is affected
  • GI endoscopy, if the gastrointestinal tract is involved
  • Skin or lymph node biopsy: A skin or lymph node biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis
  • A differential diagnosis may be used to eliminate other tumor types

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of African Endemic Kaposi Sarcoma?

The possible complications due to African Endemic Kaposi Sarcoma include:

  • Metastasis of KS to other vital body organs; the entire body could be affected leading to fatal results
  • KS can recur, even after the full treatment course is completed
  • Lung complications, with shortness of breath and bloody coughs
  • If the skin lesions are visible, then they may present cosmetic issues in individuals
  • Secondary bacterial or fungal skin infections may develop if the lesions ulcerate and bleed
  • Damage to vital nerves, blood vessels, and surrounding structures during surgery to remove the tumor
  • Side effects from chemotherapy (such as toxicity) and radiation therapy

How is African Endemic Kaposi Sarcoma Treated?

There is no definitive cure for Kaposi Sarcoma, since it is caused by a virus (HHV8); the signs and symptoms can only be controlled. The treatment depends upon the location and extent of spread of the tumors. A combination of chemotherapy, radiation therapy, and invasive procedures are used to treat KS.

Treatment measures for African Endemic Kaposi Sarcoma may include the following:

  • If no signs and symptoms are observed, because the tumor is small in size, the condition may be monitored over a period of time
  • Localized Kaposi Sarcoma is usually removed through surgical excisions; for KS involving several different parts of the body, chemotherapy and immunotherapy may be employed
  • If the lesions are confined to a localized area, then radiation therapy or cryotherapy may also be useful
  • Post-operative care is important: One must maintain minimum activity levels, until the surgical wound heals
  • Post-operative follow-up care with regular screening and check-ups are important, especially to monitor for any recurrences

How can African Endemic Kaposi Sarcoma be Prevented?

African Endemic Kaposi Sarcoma may be prevented by considering the following factors:

  • Controlling some of the modifiable risk factors, such as malaria, malnutrition, and recurrent parasitic treatment, through early diagnosis and treatment
  • Regular medical screening at periodic intervals with blood tests, scans, and physical examinations are mandatory for those who have already endured the tumor; due to both its metastasizing potential and chances of recurrence. Often several years of active vigilance is necessary

What is the Prognosis of African Endemic Kaposi Sarcoma? (Outcomes/Resolutions)

  • The prognosis of African Endemic Kaposi Sarcoma is poor, since it is a very aggressive form of KS
  • However, the prognosis may be improved, if it is diagnosed early, the lesions are small in size and few in numbers, and the condition is still in its mild form

Additional and Relevant Useful Information for African Endemic Kaposi Sarcoma:

The 4 different types of Kaposi Sarcoma are known to affect 4 diverse (ethnic) groups of people; unlike other forms of cancer that affect different cells or organs, in the same individual. This particular trait of KS remains medically unexplained.

What are some Useful Resources for Additional Information?

References and Information Sources used for the Article:

Helpful Peer-Reviewed Medical Articles:

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: Oct. 19, 2015
Last updated: April 28, 2018