Kaposi's Sarcoma

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What is Kaposi's Sarcoma?
Who are at risk from Kaposi's Sarcoma?
How is KS detected?
What are staging and grading?
How is KS treated?
What are the side effects of treatment for Kaposi's Sarcoma?
What is the importance of follow up?
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What is Kaposi's Sarcoma?

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This is a disease in which the cancer is found in tissues underneath the mucous membranes that line the mouth, nose and anal canal and also under the skin.

Kaposi's sarcoma or KS as it is commonly known as, causes purple and red blisters or papules (lesions) on these areas and spreads to other organs in the body such as the liver, the lung and the intestinal tract.

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Who are at risk from Kaposi's Sarcoma?

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In the early 1980s, it used to be an extremely rare phenomenon to encounter a KS. It was found only in older men and basically patients who have been immuno-suppressed with organ transplants and mostly in African men.

Acquired immune deficiency syndrome (AIDS), having become a pandemic (a disease present worldwide) phenomena, the disease is now quite prominent and is seen very often not only in blacks but also in gay men, who have got AIDS. All these patients who are immuno-compromised have a KS which is extremely aggressive.

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How is KS detected?

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If the patient is known to have a history of high risk such as homosexuality or has got full-blown AIDS and the doctor sees the skin and the lymph nodes and finds evidences of KS which is palpable, then the doctor may order further tests to see if the patient has KS. Also, he may try to find out if there are any other diseases or infections associated.

 
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What are staging and grading?

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These are terms with which you must become familiar, if you are dealing with any form of cancer.

When we stage a cancer, we try to establish carefully degree of spread of the cancer and if indeed there is spread, to what extent and involving which organs.

The grading of a cancer is a microscopic issue, in which the pathologist lets you know how aggressive this particular type of cancer is ; well differentiated means less aggressive, moderately differentiated intermediately aggressive and poorly differentiated more aggressive.

Both grading and staging help your clinician to establish the prognosis or how serious your disease is and what is going to be the likely outcome.

Staging may require additional imaging tests such as CT Scan, MRI, Sonography of the abdomen, Bone scan or chest X-ray.

No acceptable system of staging and grading for Kaposi's sarcoma exists. People are basically grouped into three types of KS.

Classic KS occurs in older men of Mediterranean heritage or who are Italian. This type of KS progresses slowly, sometimes over many years. As the disease spreads, the legs may swell up considerably and the blood may not flow properly. Subsequently, the disease spreads to other organs and these patients also have a predilection of developing second cancers.

Immune and successive treatment related KS is usually seen in those patients whose immune system is weakened because of the particular drugs they take to suppress the immune system. (The immune system is that system of the body which helps fight off infection). Drugs given to suppress the immune system postoperatively are classically used in patients who have undergone an organ transplant such as a heart transplant, a liver transplant or a kidney transplant. These drugs are given in order to prevent the foreign organ from being attacked by the native organs, which may think that it is foreign in nature and does not belong in the place which it now occupies.

Epidemic KS is seen in those patients who have got AIDS. These patients also may have other infections and KS in these patients is aggressive.

Recurrent KS This is a disease which has come back after the primary therapy for cancer is over. It may recur in the original place or in other parts of the body.

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How is KS treated?

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A lot depends on the patient's constitution, the grade of his disease and its staging. Patients are usually treated by a team of specialists which utilizes a multipronged approach. This includes an oncosurgeon and a radiotherapist. The plan of treatment is tailored to fit a patient's requirements. If necessary, chemotherapy and biological therapy may become add ons.

Treatment strategies

The bastions of treatment for KS are surgery, chemotherapy, radiation and biological therapy.

Radiation therapy is the commonest treatment for KS. This involves the use of high energy, penetrative rays to destroy cancer cells. It affects cancer cells only in the zone treated. Radiation therapy is also employed for palliation i.e. control of symptoms alone in an advanced cancer.

Radiation therapy can also be used in adjunct to surgery or chemotherapy, either before or after.

Teletherapy or external radiation is given via a machine remote from the body while brachytherapy or internal radiation is given by implanting a radioactive source within the involved tissues. Patients may or may not require both modalities of radiation.

Radiotherapy, after initial planning, usually takes just 5 to 10 minutes per day, 5 days a week for about 6 weeks. This time period is flexible depending upon the modality of radiation used.

Surgery may be used to take out local lesions and some of the tissues around it.

Cryotherapy or freezing method may be used to kill the tumor.

Electrodesiccation and curettage are two more methods which use a sharp instrument to clean up or scrape off the lesion after it has been burnt by electric current.

Besides local therapy, the best attempt to control cancer cells circulating in the body and lodged at places other than the original locus is by using systemic therapy (therapy which affects the entire systems of the body) which is in the form of injections or oral drugs. This form of treatment is called chemotherapy.

This form of treatment is given in a cyclical manner (each set of drugs is repeated usually after every 3-4 weeks).

Chemotherapy can also be used in combination with surgery, radiotherapy or both, either before or after.

Biological therapy employs Biological Response Modifiers (BRM's), which are substances that use the body's own immune system, either directly or indirectly, to fight cancer or to lessen the side effects of the anti cancer drugs. Some examples of BRM's are interferon-alfa and interleukin-2.

For classical KS, the following options may be entertained:
Radiation.
Local excision.
Systemic and intralesional chemotherapy.
Chemotherapy plus radiation.
For immunosuppressive treatment related KS, one of the following options may be entertained:

The cancer may be controlled if immunosuppressive drugs are stopped or if the patient cannot stop taking these drugs, then one of the following treatments may be used - radiation or chemotherapy.

For epidemic KS, one of the following treatment options are to be entertained:
Surgery in the form of local excision.
Electrodesiccation, curettage or cryotherapy with or without radiation.
Systemic chemotherapy.
Biological therapy.
New treatment methods which are being evolved by special clinical trials.

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What are the side effects of treatment for Kaposi's Sarcoma?

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Unfortunately treatment for cancer does cause damage to the healthy tissues.

Please ask the clinician about any possible side effects before any form of treatment commences.

The effects of radiation depend upon the dose of radiation, the size of the area radiated, and the number and size of each fraction.

The commonest side effect is extreme fatigue. Although bed rest is good, most radiotherapists advise the patient to be as mobile as possible.
Another common problem is that of radiation dermatitis in which the skin covering the radiated area becomes red, dry, itchy, and may show signs of scaling off. This will slowly settle down after radiation ceases, but there may be a permanent 'bronzing' of the skin. It is important to note here that this skin problem is associated only with teletherapy or external radiation therapy.

Radiation also may cause nausea and vomiting, diarrhea, urinary discomfort.
There may also be a fall in the white blood cells, which are needed by humans to fight infection.

Usually the radiotherapist can suggest the drugs and diet necessary to alleviate such problems.

The various drugs in chemotherapy cause a varied spectrum of side effects. The severity and variability of these symptoms are very individualistic, changing from patient to patient.

Some of the common side effects are:
Skin Rashes.
Loss of hair.
Diarrhea.
Vomiting.
Tingling and numbness in the fingers and toes.
Hearing loss.
Most are temporary and recede after therapy is over. Hair growth gradually starts after cessation of chemotherapy.

The medical oncologist will usually be able to reduce the severity and spectrum of these side effects.

The psychology of the patient is very important during therapy. Better psychologically prepared the patient is, better are his chances of having decreased side effects. The patient should drink large quantities of water while the therapy is in progress, and preferably avoid uncooked or raw food. Water should be filtered or boiled. Any relative or person who has any sort of infection such as common cold should be asked to desist from coming close to the patient.

Biological therapy

The side effects commonly encountered with this therapy are as follows:
Low grade fever.
Rashes and bruises.
Nausea and vomiting (usually mild).
All these problems disappear on cessation of therapy.

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What is the importance of follow up?

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The importance of followup cannot be overemphasized. It is a fatal fallacy to assume on the patient's part that once the primary or the first phase of treatment is over, then he or she is cured. It must be stressed here that cancer has a notorious proclivity of coming back again and again. The only way to fight this is to pick up any problem when it is still early, so that it can be nipped in the bud. And the only way to do this is to follow up when the doctor calls you for the next visit. Each such visit will include a detailed history, a physical examination (including an internal i.e. a rectal or vaginal examination), a chest x-ray, an ultrasonography or a CT scan, and various other blood studies as indicated.

 
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