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

• Human Herpes Virus 8 Infection causing Kaposi Sarcoma
• Kaposi's Sarcoma
• Multiple Idiopathic Pigmented Hemangiosarcoma

What is 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: It is a form of Kaposi Sarcoma that typically affects middle-aged men who belong to Mediterranean and East European descent
  • African Endemic KS: It is an unusually aggressive form of Kaposi Sarcoma that typically affects individuals of sub-Saharan African regions
  • Iatrogenic KS: It is a form of Kaposi Sarcoma that is caused by medical treatment using immunosuppressant medications, for various reasons
  • AIDS-Related KS: It is the most aggressive form of Kaposi Sarcoma that can affect any region of the body, such as the face, head, and neck region

Who gets Kaposi Sarcoma? (Age and Sex Distribution)

In general, Kaposi Sarcoma may affect any individual of any age, gender, race, or ethnicity. However, each specific type of KS affects a certain subset of individuals.

• Classic KS: It is seen in middle-aged and sometimes, older adults; males are affected more than females. Individuals of Mediterranean, East European, Middle Eastern origin, and Ashkenazi Jews have a high risk
• African Endemic KS: It is mostly seen in children and adults belonging to the sub-Saharan regions of Africa
• Iatrogenic KS: It may affect any individual with a weak or poor immunity, which is caused due to other underlying health conditions, irrespective of age, gender, race, or ethnicity
• AIDS-Related KS: Any individual who is infected with human immunodeficiency virus (HIV) may develop AIDS and be at risk for this type

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

Risk factors for Kaposi Sarcoma are again based on each specific type of KS.

• Classic KS: Those belonging to the Mediterranean, East European, or Middle Eastern region, and Ashkenazi Jews have a high risk. Also, having a family history of the condition is a risk factor
• African Endemic KS: Individuals belonging to the sub-Saharan regions of Africa are at risk; including the presence of conditions such as chronic malaria and parasitic infections, malnutrition, etc.
• Iatrogenic KS: Any medical condition necessitating treatment using immunosuppressive medications (such as an organ transplant), chemotherapy treatment, major illnesses, etc.
• AIDS-Related KS: Any individual with AIDS or HIV infection

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 Kaposi Sarcoma? (Etiology)

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.

• Classic KS and African Endemic KS: The exact cause is unknown, though genetic mutations have been observed
• Iatrogenic KS: The exact cause is unknown, though a weak immune system can trigger the condition
• AIDS-Related KS: The exact cause is unknown; however, this type is specific to individuals with AIDS

What are the Signs and Symptoms of Kaposi Sarcoma?

The signs and symptoms of Kaposi Sarcoma include:

• If the skin is affected, then KS may be manifested as a mass on the skin, which may bleed and ulcerate
• The lesions form on the body - particularly on the arms, legs, face, and groin; they are mostly painless and non-itchy
• Due to the fact that blood vessels are severely affected, the skin appearance is purple red in color
• 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
• Lymph nodes, tissues, and internal organs may be affected
• Breathing difficulty, cough, blood in sputum, and fever may be an indication that the lungs are infected

How is Kaposi Sarcoma Diagnosed?

Diagnosis of Kaposi Sarcoma is made by:

• 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 involvedSkin 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 Kaposi Sarcoma?

The possible complications due to Kaposi Sarcoma are:

• Metastasis of KS to other vital body organs; the entire body could be affected leading to fatal consequences
• 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 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.

Each form of Kaposi Sarcoma, responds to treatment in a different manner. This also depends on the health of the patient, and the individual’s specific response to treatment.

• Localized Kaposi Sarcoma is usually removed through surgical excisions; for KS involving several different parts of the body, chemotherapy and immunotherapy may be employed
• The commonly used therapy called ‘highly-active antiretroviral therapy’ (HAART) has been shown to considerably improve the condition in AIDS-affected individuals
• Decreasing or discontinuing immunosuppressive therapy may be helpful for those with Classic KS
• 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 Kaposi Sarcoma be Prevented?

Some forms of Kaposi Sarcoma may be prevented by considering the following factors:

• Adhering to safe sexual practices and avoiding multiple partners
• Controlling some of the modifiable risk factors, such as malaria, malnutrition, and recurrent parasitic treatment, through early diagnosis and treatment
• Testing organ donors for human herpes virus infection can be helpful
• Genetic counseling and genetic testing could help those individuals having a family history of the condition, planning for a child
• 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 Kaposi Sarcoma? (Outcomes/Resolutions)

• The prognosis of Kaposi Sarcoma is improved, if the condition is diagnosed early, it is still in its mild form, and when the individual does not have a poor immune system
• In general, some forms, such as African Endemic KS and AIDS-Related KS, which are more aggressive may have poorer prognoses, when compared to the lesser aggressive ones, such as Classic KS and Iatrogenic KS
• Individuals suffering from HIV infection or AIDS have the worst outcomes

Additional and Relevant Useful Information for 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.