Overview

Kaposi sarcoma was first reported by Moritz Kaposi, a dermatologist (skin specialist), in 1872. Kaposi sarcoma is caused by Kaposi sarcoma-associated virus (KSHV), also known as human herpesvirus 8 (HHV-8)¹

In this condition, cancer cells are present in the skin and mucous membranes that line the gastrointestinal tract (GI tract), which extends from the mouth to the anus and includes the stomach and intestines.

They are tumours shaped as nodules and appear as purple patches on the skin or mucous membrane that can spread to the adjacent lymph nodes or the lungs. These are more common in men and those with suppressed immune systems.²

HHV-8 infection rates vary worldwide, from as high as 40% in sub-Saharan Africa to as low as 2–4% in Northern Europe, Southeast Asia, and the Caribbean. About 10% of people in Mediterranean countries and 5–20% in the U.S. have the virus.³

What are the types of Kaposi's sarcoma?

• Classic (Mediterranean) Kaposi sarcoma –This disease has a male: female ratio of 17:1 and occurs in patients above 50 years of Eastern European and Mediterranean descent, and mostly affects the lower limbs and GI tract. They may emerge as a result of a compromised immune system^3,1
• Epidemic (AIDS-related) Kaposi sarcoma –These cases are mostly caused by HIV, which, along with KSHV, contributes to the development of Kaposi sarcoma. It is the second most frequently occurring cancer among individuals with HIV. HIV-positive men who have sex with men have a 5 to 10 times higher risk of developing Kaposi sarcoma^2,3
• Endemic (African) Kaposi sarcoma – These cases have an unusual predilection with children and are linked to HHV-8 infection. Following the HIV epidemic in Africa, the ratio of men to women with Kaposi sarcoma has fallen from 7:1 to 2:1. Endemic Kaposi sarcoma is now the most common cancer in men and the second most common cancer in women in Uganda and Zimbabwe.³ The tumour lesion is often confused with classic Kaposi’s sarcoma in the case of adults. Children often have swollen lymph nodes, lymphedema, and a fast-spreading, aggressive form of the disease that can affect internal organs¹
• Iatrogenic Kaposi/Transplant-related Kaposi sarcoma—This condition has a male: female ratio of 3:1. More than 5% of transplant patients who develop a new cancer will get Kaposi sarcoma, which is 400 to 500 times more likely than in the general population. However, solid organ transplants are more vulnerable to this type of Kaposi sarcoma as compared to those who receive bone marrow or stem cell transplants.³ Mostly presented as skin lesions¹

Indications for excision of lesions

• Excision of the lesion is considered when the lesion is small in size and is superficially placed on the skin, not reaching the deeper skin layers.⁵ This condition can only be confirmed when the lesion is studied under the microscope by a pathologist (a doctor who specialises in the study of abnormalities in tissues/fluids and organs in the body)
• Cosmetic consideration—from the cosmetic point of view, when the lesion appears unsightly, it can be excised surgically if the lesion is small and also manageable⁶
• When the lesions are small and painful, surgical excision of the nodules is indicated⁶

Types of surgery for excision of the lesion

Surgical procedures are undertaken for Kaposi’s sarcoma when it is a small skin lesion.

• Local excision: The lesion/nodule, along with a small amount of normal tissue around it, is cut from the skin for cosmetic purposes or to alleviate a painful condition 
• Electrodesiccation and curettage: A special spoon-shaped tool called a curette is used to scrape off the tumour from the skin, which is then treated with an electric current with the help of a needle-like device to stop the bleeding from the scraped portion. It also destroys any leftover cancer cells around the wound. This may be done up to three times to make sure all the cancer is removed
• Cryosurgery, or cryotherapy, is a treatment method that uses an instrument to freeze and destroy abnormal tissue
•  Excisional biopsy—a scalpel is used to remove the entire skin growth to study the lesion under the microscope in search of any cancerous signs

FAQs

What factors are responsible for increasing the risk of developing Kaposi's sarcoma? ²

• A weak immune system in the HIV-positive patient puts them at a much higher risk of being affected by Kaposi's sarcoma
• If you have undergone an organ transplantation surgery
• Your immune system may be weakened by various factors, including age or underlying health conditions

What are the symptoms of Kaposi’s sarcoma?³

• Skin lesions – purple/red/brown flat or raised lesions on skin, mostly on the feet, legs, or face
• Mucous membrane lesions
• Lesions in the body – lesions in the lungs lead to breathing problems, and you may cough up blood due to discomfort
• Swollen lymph nodes adjacent to the tumour leading to swelling of the affected organ, e.g., legs
• Lesions in the gut, lung, genitals, and lymphatic system may give rise to

• Discomfort while swallowing
• Bleeding while coughing
• Shortness of breath
• Blood in stool
• Bowel obstruction
• Swollen legs

Summary

Patients who are diagnosed with Kaposi sarcoma should be closely monitored for disease regression or recurrence and the need for radiation treatment or systemic chemotherapy.³Unlike early in the AIDS epidemic, Kaposi sarcoma is very treatable. The National Cancer Institute quoted that about 72% of people with Kaposi sarcoma are still alive five years post-diagnosis and treatment. Survival rates are increasing due to improvements in the treatment methods of the disease. It's also important to know that many people with Kaposi sarcoma die from other causes, like HIV/AIDS-related illnesses. Keep in mind, these statistics are averages. Your outlook depends on things like your age, overall health, immune system, and how far the disease has spread. Talk to your doctor for personal guidance.²