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I hope I find you in good health. A sarcoma is a cancer that develops in
connective tissues such as cartilage, bone, fat, muscle, blood vessels, or fibrous
tissues (related to tendons or ligaments). Kaposi sarcoma (KS) was named after
an Austrian dermatologist Dr. Moritz Kaposi who first described classic KS in
1872. For decades KS was considered a rare disease that mostly affected elderly
men of Mediterranean or Jewish heritage, organ transplant patients, or young adult
African men. In the last 20 years, however, the vast majority of KS cases have
developed in association with human immunodeficiency virus (HIV) infection and
the acquired immunodeficiency syndrome (AIDS), especially among homosexual men.
With the use of new treatments for AIDS, this is turning around, and the number
of KS cases due to HIV infection is decreasing. This disease typically causes
tumors to develop in the tissues below the skin surface, or in the mucous membranes
of the mouth, nose, or anus. These lesions (abnormal tissue areas) appear as raised
blotches or nodules that may be purple, brown, or red. Sometimes the disease causes
painful swelling, especially in the legs, groin area, or skin around the eyes.
Although the skin lesions of KS may be disfiguring, they usually are not life
threatening or disabling. In most cases, the lesions cause no symptoms. In some,
the lesions may be painful, especially if they cause swelling of nearby unaffected
skin. If the disease also involves the lungs, liver, gastrointestinal tract, or
lymph nodes, other symptoms may develop. KS in the gastrointestinal tract, for
example, can produce bleeding, while tumors in the lungs may cause difficulty
breathing. There are several types of KS. They each differ in their patterns
of symptoms and organs likely to be affected, how aggressively the cancer grows
and spreads, risk factors, and other personal characteristics of patients. The
treatment used and the likelihood of survival depend on the type of KS, as well
as other factors discussed later in this document. Classic Kaposi sarcoma
usually develops in Jewish men of Eastern European origin or among men of Mediterranean
heritage (primarily Italian) between the ages of 50 and 70. Classic KS is quite
rare, even in these ethnic and age groups. African (or endemic) Kaposi sarcoma
is a form of the disease that develops in people living in equatorial Africa.
This disease is fairly common. It accounts for 9% of all the cancers seen among
Ugandan men, for example. In many cases, this disease is identical to classic
KS, although it usually strikes at a much younger age. It affects many more men
than women. Typically, African (endemic) KS causes skin lesions that do not produce
symptoms and do not spread to other parts of the body. Transplant-related (or
acquired) Kaposi sarcoma refers to the form of the disease developed by people
whose immune systems have been suppressed following an organ transplant. Usually
a transplant patient must take drugs to prevent the immune system from "recognizing"
the newly transplanted organ as foreign to the body. Because these drugs weaken
the body's defenses, other diseases or infections can take hold. AIDS-related
(or epidemic) Kaposi sarcoma arises in people who are infected with HIV. This
is probably the commonest in Zimbabwe. It was in part the unusual and sudden appearance
of this form of KS in so many young men at the start of the AIDS epidemic that
led doctors to realize that a new disease had emerged. Acquired immune deficiency
syndrome (AIDS) results from infection by the human immunodeficiency virus (HIV).
This virus destroys certain cells of the immune system, making the body unable
to fight infections caused by certain other viruses, bacteria, and parasites.
Certain forms of cancer are also more likely to develop in people whose immune
systems have been damaged. A person infected with HIV (that is, being HIV-positive)
does not necessarily have AIDS. The virus can be present in the body for a long
time, typically many years, before causing any major illness. The disease known
as AIDS begins when the virus has caused serious damage to the immune system,
which results in certain types of infections and other medical complications.
Certain diseases occur so often in people with AIDS that they are considered
"AIDS-defining conditions" -- that is, their presence in a person infected
with HIV is a clear sign that full-blown AIDS has developed. Certain cancers have
been identified as AIDS-defining diseases e.g. Kaposi sarcoma, lymphoma (especially
non-Hodgkin lymphoma and primary central nervous system lymphoma), anal cancer,
and cancer of the cervix that has spread to neighboring tissue (called invasive
cervical cancer). Many other kinds of cancer may be more likely to develop in
people with HIV infection. Of course, people without HIV or AIDS can also have
these types of cancer. In most cases, epidemic KS causes widespread lesions
that erupt at many places on the body soon after AIDS develops. Lesions of epidemic
KS may arise on the skin and the mouth and may affect the lymph nodes and other
organs, usually the gastrointestinal tract, lung, liver, and spleen. In contrast,
classic KS usually involves only one or a few areas of skin, most often the lower
legs. At the time of diagnosis, some people with epidemic KS experience no symptoms,
especially if their only lesions develop on the skin. However, many even those
with no skin lesions will have swollen lymph nodes, unexplained fever, or weight
loss. Eventually, in almost all cases, epidemic KS spreads throughout the body.
Extensive lung involvement with KS can be fatal. More often, however, patients
die of other AIDS-related complications such as infections. Modern treatment of
the HIV infection with antiretroviral drugs usually prevents the development of
advanced Kaposi sarcoma.
KS is not considered curable. Neither surgical
removal of the first-detected lesion nor obtaining a complete remission of multiple
sites with chemotherapy or other techniques results in cure. Long-term survival
does occur both with or without treatment, however. Survival in classic KS
is usually years and sometimes decades. Some patients with AIDS-related KS are
still alive after 10 years, though most survive only a few years and treatment
decisions are usually aimed at palliation. All forms of KS are sensitive to
radiation therapy. Radiation is especially useful for lesions that are cosmetically
disturbing, painful, involve the mouth extensively, block lymphatics, bleed, or
protrude from the skin. Response rates are quite high and treatment is well tolerated.
Chemotherapy can be used in treatment but there is concern that aggressive
treatment might further depress the immune system. The disease does respond to
chemotherapy, both with single agents and combinations of drugs. KS is one
of the few tumors that responds to local injections of chemotherapy. Many anticancer
drugs cause intense local damage if they are accidentally injected into tissue.
This undesirable effect has been used in a positive way to treat skin lesions The
prognosis (likelihood of survival) for patients with classic KS is good. Tumors
can frequently be controlled and patients frequently die of other causes before
any serious spread. African endemic KS can progress rapidly and lead to premature
death, despite treatment. In AIDS-related KS, milder cases can frequently be controlled;
the prognosis for more advanced and rapidly progressing cases is less certain
and dependent on the patient's overall medical condition. There are indications
that KS can be stabilized or reversed in patients whose level of HIV in the blood
is reduced to undetectable levels via antiretroviral therapy. Safer sex practices
may help to prevent AIDS-related KS by decreasing the risk of transmission of
HHV-8. Treatment with antiretrovirals and protease inhibitors may help to preserve
the function of the immune system in HIV patients and delay the appearance and
progression of KS lesions.
Till next week take care of yourself and
each other. Remember, "
in all your getting, get understanding."
Dr
P. Chimedza is a medical practitioner with a special interest in HIV and AIDS.
Information for this article was researched from different medical textbooks,
medical journals and other medical information sources for which this author hereby
acknowledges. This column is kindly sponsored by Generation Health. For further
information on this topic and suggestions on future topics for discussion you
can mail him on pchimedza@hotmail.com.
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