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Kaposisarcoma

KAPOSI SARCOMA
Kaposi sarcoma (KS) is a rare type of soft tissue sarcoma which normally develops below the skin surfaces or on the mucosal lining of other body parts such as mouth, anus, nose, throat etc. This type of sarcoma can also develop in the lymph nodes. Sarcoma is a type of cancer that affect supportive and connective tissues of the body.
When this type of cancer develop under the skin, abnormal tissue changes will appear on the skin surface. These abnormal changes of tissues on the skin are known as lesions. Depending on skin color of a person, the lesions normally appears as brown, purple or red. Statistics indicate that, men are more affected by this cancer than women.

Types of Kaposi sarcoma
There are mainly four types of Kaposi sarcoma. These types are;
• Epidemic Kaposi sarcoma. Epidemic Kaposi sarcoma is a type of KS that develop to people with Acquired immune deficiency syndrome (AIDS). This type of KS can develop and form lesions from different parts of the body such as lymph nodes, lungs, liver and spleen. Although nowadays the number is decreasing, but this type of KS, still contribute a significant number of cases to total number of KS patients in Tanzania.
• Classic Kaposi sarcoma. This type of Kaposi sarcoma normally occurs to older men of Jewish or Mediterranean descents. This type of cancer normally develops to the lower parts of the body including legs and ankle.
• Endemic Kaposi sarcoma. Endemic Kaposi sarcoma is a type of sarcoma that is found in some parts of Africa. This type of KS is common to those living in equatorial Africa. This type of Kaposi sarcoma tend to occur in younger people and normally below 40 years. Aggressive form of endemic Kaposi sarcoma is found to children before puberty.
• Iatrogenic (acquired) Kaposi sarcoma. It is a type of Kaposi sarcoma that develop in people using immune suppressive drugs after organ transplantation to prevent their body from rejecting the transplanted organ.
Those are the main types of Kaposi sarcoma. However, there are concerns that there is another type of Kaposi sarcoma that develop to men who have sex with men. This type of KS affects men having sex with men who are HIV negative.

Causes of Kaposi sarcoma
Kaposi sarcoma is mainly caused by Kaposi sarcoma herpes virus (KSHV) infection. This virus is also known as Human herpesvirus 8 (HHV8). Most people with this virus never get KS, because for them to develop the disease, their immune system has to be weak or weakened.
Our immune system is made up of tissues and organs that work together to protect us against infections. Once the immune system is weakened and you have this type of virus is when infection occurs. For example; people with HIV infection and those using immunosuppressive drugs after organ transplantation.

Risk factors of Kaposi sarcoma
When it comes to cancer, anything that increases your probability of developing cancer is called a risk factor. Risk factors only influence development of cancer but it does not directly cause cancer. Sometimes people with risk factors do not develop the disease while those with no risk factors do develop the disease. Knowing risk factors will help you live your life making some better healthy choices to reduce the risk. The following factors may increase your risk of developing Kaposi sarcoma.
• Gender. Statistics indicate that, number of men who develop Kaposi sarcoma is higher compared to that of women.
• Ethnicity. Some types of Kaposi sarcoma only develop to people who live in a certain area. For example, endemic sarcoma that develops to those living in equatorial Africa and classic KS that develop to those Mediterranean descents.
• Having AIDS. Those with AIDS are at increased risk of developing Kaposi sarcoma as their body immunity is low. When these people comes into contact with KSHV, it is easy for them to be infected and develop Kaposi sarcoma.
• Sexual practices. Men who are homosexuals are said to be at an increased risk of KSHV infection hence development of Kaposi sarcoma.

Prevention of Kaposi sarcoma
In order to prevent something, you need first to know what causes it. It is unfortunate for Kaposi sarcoma, because we know the cause but we still don`t have a way to completely prevent it. Kaposi sarcoma is caused by Kaposi sarcoma human virus (KSHV) also known as human herpesvirus 8 (HHV8). Currently there are no vaccines for this virus hence preventing development of this cancer depends on reducing the risk for developing this cancer.
The risk can be reduced through the following;
• Avoiding sexual practices that puts you at increased risk of HIV infection.
• Those with HIV infection should take ant-retroviral drugs (ARV) drugs so as to try to strengthen their body immunity.
• Since men having sex with men is said to increase risk of developing KS, then men are advised to avoid this practice.

Early detection and screening of Kaposi sarcoma
When it comes to cancer, early detection normally provide better treatment outcome. Although most cases of Kaposi sarcoma have good treatment outcome, chances for better treatment outcome will be very high if the disease is found at a very early stage. Early detection of cancers, have been greatly contributed by screening. Screening is the process of running some tests to someone with no symptoms of a certain disease with the intention of determining presence or absence of that disease. For several other types of cancers, screening has helped in their early detection. Currently there is no widely recommended screening tests for Kaposi sarcoma. But for those at very high risk of developing Kaposi sarcoma like those infected with HIV, are recommended to have regular examinations by doctors experienced in recognizing KS.

Signs and symptoms of Kaposi sarcoma
Signs and symptoms of Kaposi sarcoma depends on the site where the disease has developed. The signs and symptoms of Kaposi sarcoma include the following;
• Skin lesions. The lesions normally appears as brown, purple or red bump or nodule or sometimes just a flat skin surface. Color of a bump may vary depending on the skin color of a person.
• If KS develop in the lungs it can cause breathlessness or cough
• KS in the digestive system (stomach and bowel) can cause someone to feel nausea and sometimes vomiting
• KS can affect lymph nodes and cause swelling. The swelling will depend on the location of the affected nodes but normally it causes swelling of legs or arms.
• Anemia. Sometimes there will be slow bleeding from the lesions, but if this persist over a period of time, it may lead to low red blood cells count (anemia).
• Lesions in the mucosal lining of different parts of the body such as mouth, eyelids and throat. These lesions are normally not painful.

Diagnosis of Kaposi sarcoma
cancer diagnosis
Medical diagnosis simply means identification of nature of an illness by elimination of the symptoms. Normally people don`t go to hospital knowing they have cancer but during diagnostic procedures is when doctors detect the disease. Following suspicion of the disease, doctors normally run combination of tests to determine whether there are cancerous cells in the body or not and if there are cancer cells, how far have they spread. Along with thorough medical history and physical examination, doctors normally use combination of the following tests to diagnose cancer.
Laboratory tests.
Laboratory tests are important in ruling out other conditions and confirming diagnosis. For cancer diagnosis laboratory tests normally include blood tests, urine tests, tumor markers and other body fluids tests.
• Blood tests. Blood tests help to reveal level of different substances in the blood which will help doctors to know if there is anything wrong in the body. From blood tests doctors can see complete blood count which indicates number, size and maturity of blood cells. Also from the blood tests doctors can analyze if kidney and liver are working properly.
• Urine tests. Urine tests (urinalysis) involve laboratory examination of urine to check presence of blood, proteins, and other substances such as drugs. For instance, blood in the urine may be an indication of benign condition, infection or other health problem.
• Tumor markers. These are biomarkers (proteins) that can be produced by both cancer cells and normal body cells in response to cancer. Tumor markers are released into the blood or urine. Although studies are still conducted to determine role of these markers in diagnosing cancer because sometimes people with benign condition will have elevated level of these proteins. Also not all tumors have their markers and some markers are not specific to one type of tumor.
• Other body fluids. Despite being rarely used, sometimes body fluids can be tested in the lab during diagnosis of cancer. For instance, the use of cerebral spinal fluid (CSF) and pleural fluid. CSF is used in diagnosis of brain tumors while pleural fluid for lung cancer.

Diagnostic imaging
Diagnostic imagings involve tests that create pictures of inside the body that help the doctor to see if there is tumor or not. There are several imaging tests used in cancer diagnosis depending on the type and location of cancer suspected. These imaging tests include;,
• Computed tomography (CT) scan. CT scan machine is a donut-shaped scanner that uses x-rays to create series of pictures of body organs from different angles. This machine is linked to a computer where those series of pictures taken will be combined to create a 3D- image of inside the body. During the scanning process patient will lie still on the table which will slide into the scanner. In order to get a clear image, sometimes the doctor will inject the patient with a contrast material before scanning. From the images the doctor will be able to differentiate between healthy and unhealthy tissues.
• Magnetic resonance imaging (MRI). MRI is an imaging technique that uses powerful magnet and radio waves to take series of pictures of body organs and create a 3D image in a computer linked to a machine. Just like in CT, during MRI procedure the patient will lie on a table that will slide into a long round chamber. Also during this procedure, the doctor may inject the patient with a contrast material for clear image view.
• X-ray. This is an imaging technique that is uses low dose of radiation to create pictures of inside your body. Technician will position the patient and direct the x-ray beam to the intended part of the body.
• Ultrasound. Ultrasound is a diagnostic imaging procedure that uses high energy sound waves to produce images of organs inside the body. During the examination, a patient will lie on the table while the technician will slowly move a device called transducer on the skin over the part of the body that is being examined.
• PET scan. Positron emission tomography (PET) scan is an imaging test that uses radioactive glucose to create 3-D images of areas inside the body. Radioactive glucose is used for this test because cancer cells tend to absorb more glucose than normal body cells, so the scan will show which areas of the body has more glucose than others. Before the scan, doctor will inject the patient with tracer called radioactive glucose then during imaging the patient will be asked to lie on the table that will be moving back and forth through the scanner.
• Bone scan. Bone scan is used to examine bones. When it comes to cancer, bone scan is used for diagnosis of bone cancer or cancer that has metastasized into the bones. Before the test, patient will be injected with small amount of radioactive material that tends to collect more on abnormal parts of the bones. Then pictures that will be taken by a scan will indicate the distribution of those radioactive materials in the bones throughout the body.

Biopsy
Biopsy is a diagnostic procedure that involves removing a tissue sample from the body and examines it under microscope in the laboratory. Examination in the laboratory is normally done by a pathologist who will check if the cells in the tissue are cancerous or not. In most cancer cases, biopsy is considered to be the confirmatory test. Depending on the location and type of cancer, biopsy can be obtained in different ways. Some of them include;
• Using needle. By using a thin needle, doctors can draw same tissue or fluid for examination under microscope. This method can be used to draw some fluid (spinal tap), bone marrow (bone marrow aspiration), blood or small amount of tumor from the suspected organ such as liver and breast (fine needle aspiration).
• Surgery. Abnormal tissue samples are obtained while the doctor is performing the surgery. Tissues are then sent to the lab for examination during the surgery. If the surgeon removes just a portion of abnormal area, it is called incisional biopsy while when the entire tumor (abnormal site) is removed it is called excisional biopsy. • Using endoscope. Endoscope is a thin, lighted tube with a camera (focusing telescope) at the end. It is used for viewing inside the body through natural openings like mouth and anus. During an examination if the doctor sees any abnormal tissue, then endoscope can also be used to take sample for that tissue. There are different kinds of endoscopy exams depending on the site of the body being examined. Some of the exams include colonoscopy (for rectum and colon), bronchoscopy (for trachea, bronchi and lungs) and esophagogastroduodenoscopy EGD (for esophagus, stomach and duodenum)
In addition to medical history and physical examination, diagnosis of Kaposi sarcoma normally involves the use of CT scan, MRI scan, Endoscopy, Bronchoscopy, biopsy and simple photography whereby if the disease has affected the skin, by taking pictures of the affected part regularly may help the doctors notice if there is any new lesion or not.

Staging of Kaposi sarcoma
Staging is a standard way used by cancer care team to explain how far the cancer has grown or spread. Once an imaging modality has helped to establish a probable diagnosis of Kaposi sarcoma, the next issue is deciding suitable treatment option for the disease. Staging has enabled oncology team to decide on the suitable treatment option. In this process of staging doctors normally use systems in order to describe the stage of cancer.
Unfortunately, for Kaposi sarcoma, there is no staging system which is officially accepted like there is for most other forms of cancer. But for AIDS-related KS, most doctors use the AIDS Clinical Trials Group system (ACTG). The system was developed in 1988 in America. This system evaluates the disease as follows;
• Tumor (T). Describe tumor size.
• Immune system (I). Immunity status is measured by checking number of special type of white blood cells called CD4.
• Systemic illness (S). This explains the spread of the disease throughout the body or the presence of HIV/AIDS-related systemic illness.
Under each category of the TIS system (tumor, immunity system and systemic illness), there are two groups, the good risk and poor risk group. Description of the system is as shown in the table below.

TIS system

# Tumor(T) At poor risk(T1) At good risk(T0)
1 -There is ulcerations or swelling due to tumor.-Tumor has spread to other organs such as lung, liver and intestine.- There is extensive oral tumor. Lesions are nodular or found in other areas other than palate. Tumor is only on the skin and/or lymph nodes.Tumor is located on the palate (floor of the mouth)Tumors on the mouth is flat and not nodular (raised)  --
2 Immune system (I)  (I1)CD4 cell count is less than 150 per mm3  (I0)CD4 cells count more than 150 per mm3
3    Systemic illness (S)           (S1)There are other HIV related diseases. KPS is less than  70Experiencing one or more B symptoms such as (unexplained fever, diarrhea, weight loss)                   (S0)There are no HIV relates diseases.KPS is higher than 70There are no B symptoms

KPS is an abbreviation of Karnofsky Performance Status which measures the ability of people with cancer to perform ordinary tasks. For example, people with a score of 70 can take care of themselves but are unable to carry on normal activity or active work.
After assessment of the above three tumor, immunity and systemic illness status, your overall group can be determined. Because of the use of ant-retroviral drugs, immunity status is considered to be less important and is not included most of the times. Hence you can be grouped either in good risk group or poor risk depending on your status. The groups will be as follows;
• Good risk: T0 S0, T1 S0, or T0 S1
• Poor risk: T1 S1.

Treatment of Kaposi sarcoma
Following cancer diagnosis, the doctor will break the news and it is normally not very easy to accept that it is cancer. The doctor will be there to help the patient process the news and to help the patient in taking the next steps.
After the patient receives and processes the news that he/she has cancer, one of the most important steps that will follow is decision making. The doctor will present to the patient (relatives/parents/guardians) with all the possible treatment options and explain what they mean. Then with the help of the doctor, the patient (relatives/parents/guardian) will have to choose which treatment option is good depending on benefits of such option. Despite all the information given by the doctor, sometimes the patient may still be unsatisfied with what he/she has been told. In such situation, the patient is free and advised to seek a second opinion. Second opinion is the opinion that the patient may seek from another specialist (doctor) regarding his/her health problem and in this case it is cancer. Second opinion may include confirmation of the diagnosis, more clarification on your primary doctor recommendations or even reassurances that all the options have been explored. In addition to standard care treatment options, sometimes there are those new treatments or drugs or combination of treatments that have not been approved yet to be used as standard care treatments for a particular cancer (disease) but have shown some promising results that they may help. These type of treatments are called clinical trials.,
If there are some clinical trials for your case, the doctor may present to the patient or the patient may ask if there are any clinical trials for the particular cancer case. Over the years, have improved treatments and led to better outcomes to people diagnosed with various diseases including cancer. If you have decided to take part in clinical trial, you can also withdraw at any time

Treatment options
Treatment of Kaposi sarcoma requires a team of doctors and other health care professionals. This team work together and it is called multidisciplinary team. Depending on your treatment option, Kaposi sarcoma treatment team may include, dermatologist, surgical oncologist, medical oncologist, radiation oncologist, infectious disease specialist, oncology nurses, palliative care team and other health care professionals. Treatment options for Kaposi sarcoma cases include the following;
Surgery
Surgery is the treatment option that involve removing cancer cells and surrounding healthy cells through by operation. Surgery is performed by surgical oncologist. For most cases of Kaposi sarcoma, surgery is not an option because surgery is good for cases with only few and small lesions and most cases of KS have multiple lesions. Surgery for KS cases may be done in the following ways;
• Simple excision. This involve removing the tumor by cutting it out with a surgical knife. The skin in a treatment area is numbed by local anesthesia before the procedure.
• Curettage and electrodissection. In this option, tumor is removed by a sharp, spoon-shaped instrument called a curette. The area can then be treated with electrodessication, which uses an electric current to control bleeding and destroy any remaining cancer cells.

Ant-retroviral treatment
This is a treatment option that is used to those with epidemic Kaposi sarcoma. Patients with this type of KS, normally receive antiretroviral treatment (ART) first before any other treatment so as to reduce the symptoms. ART may be given alone or in combination with chemotherapy depending on patient`s symptoms and spread of the disease. One of the possible side effects of this treatment is the possibility of causing preexisting infections and the Kaposi sarcoma worse. A reaction is called immune reconstitution inflammatory syndrome (IRIS).

Biological therapy
Biological therapy is the treatment option that involve the use of drugs to boost natural immune system of the body to fight of the disease. This therapy is also known as immunotherapy. It uses materials made either by the body or in a laboratory to improve, target, or restore immune system function.
Depending on the type of drug used, possible side effects of immunotherapy may include fever, pain, weakness, low blood cell count and confusions.

Radiation therapy
Radiation therapy is an option that involves using high energy x-rays or other radiation particles to destroy cancer cells. This treatment option is provided by radiation oncologist. When dealing with KS, the most common used type of radiation therapy is external beam radiotherapy. External beam radiotherapy involves directing radiations to the tumor from the source in the machine positioned outside the body.

Side effects of radiation therapy
Side effects of radiation therapy will depend on the location of irradiation site and dose given. Most common side effects include;
• Skin reactions
• Loss of appetite
• Nausea and/or vomiting
• Fatigue

Chemotherapy
Chemotherapy is the treatment option that involve the use of drugs to kill or stop development of cancer cells. This treatment option is given by medical oncologist. When dealing with Kaposi sarcoma, chemotherapy may be given in different ways depending on the purpose.
• Systemic chemotherapy. This is used when tumor is not in one place (local). During this therapy, drugs are given either intravenously (as injections) or orally (as pill or capsule).
• Intralesional chemotherapy. In this is used for local tumors whereby the drug is injected directly into the lesion.

Side effects of chemotherapy
Side effects of chemotherapy depends on type of drugs given, dose and periodic time the by which the drug has been used. Some of the possible side effects include the following;
• Mouth sores
• Hair loss
• Loss of appetite
• Feeling tired due decreased red blood cells
• Risk of infections due to low white blood cells
• Easily bruised and bleeding
• Nausea and/or vomiting.

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