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Kaposi sarcoma is a type of cancer characterized by the abnormal growth of blood vessels that develop into skin lesions or occur internally. Learn about Kaposi sarcoma and find information on how we support and care for people with Kaposi sarcoma before, during, and after treatment.
When you come to the Center for Sarcoma and Bone Oncology,
you'll meet with members of our team who have expertise in caring for patients with sarcoma.
Patients with sarcoma often require a combination of surgery,
chemotherapy, and radiation therapy. We recognize that a team approach is the best
way to manage these complicated cases.
This means pathologists, medical oncologists, radiologists,
surgeons and other health care professionals who specialize in sarcoma may be
involved in decisions about your care.
Our group is also dedicated to clinical research to develop innovative
treatment strategies for soft tissue and bone malignancies.
We will work with you to find other support services within
Dana-Farber, including nutrition, complementary therapies, spiritual support,
financial help, survivorship, and resources for families and young adults.
Our specialists see patients with all sarcomas and a variety
of mesenchymal tumors, including:
If you have never been
seen before at Dana-Farber/Brigham and Women's Cancer Center,
please call 877-442-3324
or use this online form
to make an appointment.
If you need to schedule a follow-up appointment
or for other questions, you’ll find your clinician’s contact information here
Learn more about the Center for Sarcoma and Bone Oncology
Kaposi sarcoma is a cancer that causes lesions (abnormaltissue) to grow in the skin; the mucous membranes lining the mouth, nose, and throat; lymph nodes; or other organs. The lesions are usually purple and are made of cancer cells, new blood vessels, red blood cells, and white blood cells. Kaposi sarcoma is different from other cancers in that lesions may begin in more than one place in the body at the same time.
Human herpesvirus-8 (HHV-8) is found in the lesions of all patients with Kaposi sarcoma. This virus is also called Kaposi sarcoma herpesvirus (KSHV). Most people infected with HHV-8 do not get Kaposi sarcoma. Those infected with HHV-8 who are most likely to develop Kaposi sarcoma have immune systems weakened by disease or by drugs given after an organ transplant.
There are several types of Kaposi sarcoma, including:
The following tests and procedures may be used:
One of the following types of biopsies may be done to check for Kaposi sarcoma lesions in the skin:
An endoscopy or bronchoscopy may be done to check for Kaposi sarcoma lesions in the gastrointestinal tract or lungs.
The following tests and procedures may be used to find out if cancer has spread to other parts of the body:
The prognosis (chance of recovery) and treatment options depend on the following:
Classic Kaposi sarcoma is a rare disease that gets worse slowly over many years.
Patients may have one or more red, purple, or brown skin lesions on the legs and feet, most often on the ankles or soles of the feet. Over time, lesions may form in other parts of the body, such as the stomach, intestines, or lymph nodes. The lesions usually don't cause any symptoms, but may grow in size and number over a period of 10 years or more. Pressure from the lesions may block the flow of lymph and blood in the legs and cause painful swelling. Lesions in the digestive tract may cause gastrointestinal bleeding.
Some patients with classic Kaposi sarcoma may develop another type of cancer before the Kaposi sarcoma lesions appear or later in life. Most often, this second cancer is non-Hodgkin lymphoma. Frequent follow-up is needed to watch for these second cancers.
African Kaposi sarcoma is a fairly common form of the disease found in young adult males who live near the equator in Africa. Signs of African Kaposi sarcoma can be the same as classic Kaposi sarcoma. However, African Kaposi sarcoma can also be found in a much more aggressive form that may cause sores on the skin and spread from the skin to the tissues to the bone. Another form of Kaposi sarcoma that is common in young children in Africa does not affect the skin but spreads through the lymph nodes to vitalorgans, and quickly becomes fatal.
This type of Kaposi sarcoma is not common in the United States and treatment information is not included in this summary.
Immunosuppressive therapy–related Kaposi sarcoma is found in patients who have had an organtransplant (for example, a kidney, heart, or liver transplant). These patients take drugs to keep their immune systems from attacking the new organ. When the body's immune system is weakened by these drugs, diseases like Kaposi sarcoma can develop.
Immunosuppressive therapy–related Kaposi sarcoma often affects only the skin, but may also occur in the mucous membranes or certain other organs of the body.
This type of Kaposi sarcoma is also called transplant-related or acquired Kaposi sarcoma.
Epidemic Kaposi sarcoma occurs in patients who have acquired immunodeficiency syndrome (AIDS). AIDS is caused by the human immunodeficiency virus (HIV), which attacks and weakens the immune system. When the body's immune system is weakened by HIV, infections and cancers such as Kaposi sarcoma can develop.
Most cases of epidemic Kaposi sarcoma in the United States have been diagnosed in homosexual or bisexual men infected with HIV.
The signs of epidemic Kaposi sarcoma can include lesions in different parts of the body, including any of the following:
Kaposi sarcoma is sometimes found in the lining of the mouth during a regular dental check-up.
In most patients with epidemic Kaposi sarcoma, the disease will spread to other parts of the body over time. Fever, weight loss, or diarrhea can occur. In the later stages of epidemic Kaposi sarcoma, life-threatening infections are common.
HAART (highly active antiretroviral therapy) is a combination of several drugs that block HIV and slow down the development of AIDS and AIDS-related Kaposi sarcoma. For information about AIDS and its treatment, see the AIDSinfo Web site.
There is a type of nonepidemic Kaposi sarcoma that develops in homosexual men who have no signs or symptoms of HIVinfection. This type of Kaposi sarcoma progresses slowly, with new lesions appearing every few years. The lesions are most common on the arms, legs, and genitals, but can develop anywhere on the skin.
This type of Kaposi sarcoma is rare and treatment information is not included in this summary.
RecurrentKaposi sarcoma is cancer that has recurred (come back) after it has been treated. The cancer may come back in the skin or in other parts of the body.
Different types of treatments are available for patients with Kaposi sarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
For the treatment of epidemic Kaposi sarcoma, highly active antiretroviral therapy (HAART) is used to slow the progression of AIDS. HAART may be combined with anticancer drugs and medicines that prevent and treat infections.
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of cancer being treated.
Certain types of external radiation therapy are used to treat Kaposi sarcoma lesions. Photon radiation therapy treats lesions with high-energy light. Electron beam radiation therapy uses tiny negatively charged particles called electrons.
The following surgical procedures may be used for Kaposi sarcoma to treat small, surface lesions:
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). To treat local Kaposi sarcoma lesions, such as in the mouth, anticancer drugs may be injected directly into the lesion (intralesional chemotherapy). Sometimes the chemotherapy is given as a topical agent (applied to the skin as a gel.) The way the chemotherapy is given depends on the type of cancer being treated.
Liposomal chemotherapy uses liposomes (very tiny fat particles) to carry anticancer drugs. Liposomal doxorubicin is used to treat Kaposi sarcoma. The liposomes build up in Kaposi sarcoma tissue more than in healthy tissue, and the doxorubicin is released slowly. This increases the effect of the doxorubicin and causes less damage to healthy tissue.
See Drugs Approved for AIDS-Related Kaposi Sarcoma for more information.
Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Interferon alfa is a biologic agent used to treat Kaposi sarcoma.
Information about clinical trials is available from the NCI Web site.
Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibodytherapy is one type of targeted therapy being studied in the treatment of Kaposi sarcoma.
Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. These may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells.
Bevacizumab is a monoclonal antibody that is being studied in the treatment of Kaposi sarcoma.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment for single lesions may include the following:
Treatment for lesions all over the body may include the following:
Treatment for Kaposi sarcoma that affects lymph nodes or the gastrointestinal tract usually includes chemotherapy with or without radiation therapy.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with classic Kaposi sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI Web site.
Treatment for immunosuppressive therapy–related Kaposi sarcoma may include the following:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with immunosuppressive treatment related Kaposi sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI Web site.
Treatment for epidemic Kaposi sarcoma may include the following:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with AIDS-related Kaposi sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI Web site.
Treatment for recurrentKaposi sarcoma depends on which type of Kaposi sarcoma the patient has. Treatment may include a clinical trial of a new therapy.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent Kaposi sarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI Web site.
For more information from the National Cancer Institute about Kaposi sarcoma, see the following:
For general cancer information and other resources from the National Cancer Institute, see the following:
This information is provided by the National Cancer Institute.
This information was last updated on June 12, 2014.
Kaposi sarcoma (KS) was first described in 1872 by the Hungarian
dermatologist, Moritz Kaposi. From that time until the current human
immunodeficiency virus (HIV) disease epidemic identified with the Acquired
Immunodeficiency Syndrome (AIDS), KS remained a rare tumor. While most of the
cases seen in Europe and North America have occurred in elderly men of Italian
or Eastern European Jewish ancestry, the neoplasm also occurs in several other
distinct populations: young black African adult males, prepubescent children,
renal allograft recipients, and other patients receiving immunosuppressive
therapy. The disseminated, fulminant form of KS associated with HIV disease is
referred to as epidemic KS to distinguish it from the classic, African, and
transplant-related varieties of the neoplasm. In addition, KS has been
identified in homosexual men apart from the HIV disease epidemic.
the histopathology of the different types of the Kaposi tumor is essentially
identical in all of these groups, the clinical manifestations and course of the
disease differ dramatically. A key piece to the puzzle of KS pathogenesis
was the 1994 discovery of a gamma herpes virus, human herpes virus type 8
(HHV-8), also known as Kaposi sarcoma herpes virus. HHV-8 was identified
in KS tissue biopsies from virtually all patients with classic, African,
transplant-related, and AIDS-associated KS but was absent from noninvolved
Considered a rare disease, classic KS occurs more often in males, with a ratio
of approximately 10 to 15 males to 1 female. In North Americans and Europeans,
the usual age at onset is between 50 and 70 years. Classic KS tumors
usually present with one or more asymptomatic red, purple, or brown patches,
plaques, or nodular skin lesions. The disease is often limited to single or
multiple lesions usually localized to one or both lower extremities, especially
involving the ankles and soles.
Classic KS most commonly runs a
relatively benign, indolent course for 10 to 15 years or more, with slow
enlargement of the original tumors and the gradual development of additional
lesions. Venous stasis and lymphedema of the involved lower extremity are
frequent complications. In long-standing cases, systemic lesions can develop
along the gastrointestinal tract, in lymph nodes, and in other organs. The
visceral lesions are generally asymptomatic and are most often discovered only
at autopsy, though clinically, gastrointestinal bleeding can occur. As many as 33% of the patients with classic KS develop a second primary malignancy,
which is most often non-Hodgkin lymphoma.
In the 1950s, KS was recognized as a relatively common neoplasm endemic in
native populations in equatorial Africa and comprised approximately 9% of all
cancers seen in Ugandan males. African KS is seen as either an indolent
neoplasm identical to the classic disease seen in Europe and North America or
as an aggressive disease with fungating and exophytic tumors that may invade
the subcutaneous and surrounding tissue including the underlying bone. In
Africa, both the indolent and locally more aggressive forms of KS occur with a
male-to-female ratio comparable to that observed with the classic KS tumor seen
in North America and Europe. In general, however, patients in Africa are
significantly younger than their European counterparts. A lymphadenopathic
form of KS is also seen in Africa, primarily in prepubescent children
(male:female ratio 3:1). In these cases, the generalized lymphadenopathy is
frequently associated with visceral organ involvement. The prognosis is very
poor with a 100% fatality rate within 3 years.
In 1969, the first case of KS in association with immunosuppression in a renal
transplant patient was described. Since that time, a number of renal and other
organ allograft recipients who received prednisone and azathioprine developed
KS shortly after the onset of immunosuppressive therapy. Estimates of the incidence of
KS in immunosuppressed renal transplant recipients are between 150 and 200 times the expected incidence of the tumor in the general
population. The average time to develop KS after transplantation is 16
months. Although the KS tumor in iatrogenically immunosuppressed patients
often remains localized to the skin, widespread dissemination with
mucocutaneous or visceral organ involvement is common. In some cases, the KS
tumors have regressed as a result of reduction or changes in immunosuppressive
therapy. Clinical management of renal transplant patients who develop KS is
difficult and requires a balance between the risk of death from generalized KS
and the risk of graft rejection and complications of renal failure that may
occur if the immunosuppressive therapy is discontinued.
In 1981, a fulminant and disseminated form of KS in young homosexual or
bisexual men was first reported as part of an epidemic now known as AIDS.
The etiology of AIDS is a T-cell lymphotropic retrovirus known as HIV. The
underlying immunologic deficiency that characterizes HIV disease is an acquired
profound disorder of cell-mediated immune functions. This immunologic
deficiency and immune dysregulation predisposes the host to a variety of
opportunistic infections and unusual neoplasms, especially KS. HIV may
play an indirect role in the development of KS.
Approximately 95% of all the cases of epidemic KS in the United States have
been diagnosed in homosexual or bisexual men. In the past, approximately 26%
of all homosexual males with HIV disease presented with, or eventually
developed, KS during the course of their illness. By comparison, fewer than 3%
of all heterosexual intravenous drug users with HIV disease developed KS. The
proportion of HIV disease patients with KS has steadily decreased since the
epidemic was first identified in 1981. About 48% of AIDS patients in 1981
had KS as their presenting AIDS diagnosis. By August 1987, the cumulative
proportion of AIDS patients with KS had diminished to fewer than 20%. The
introduction of highly active antiretroviral therapy (HAART) has delayed or
prevented the emergence of drug-resistant HIV strains, profoundly decreased viral
load, led to increased survival, and lessened the risk of opportunistic
infections. The use of HAART has been associated with a sustained and substantial decline in KS incidence in multiple large cohorts.
The lesions that develop may involve the skin; oral mucosa; lymph
nodes; and visceral organs, such as the gastrointestinal tract, lung, liver, and
spleen. Most patients with HIV disease who present with the mucocutaneous
lesions of KS feel healthy and are usually free of systemic symptoms, as
compared to patients with HIV disease who first develop an opportunistic
infection. The sites of disease at presentation of epidemic KS are much more
varied than the sites seen in other types of this neoplasm. In an early report
on the clinical manifestations of the disease, 49 patients were described.
Of these patients, 8% had no skin involvement, 27% had localized or fewer than five skin
lesions, and 63% had innumerable skin lesions widely distributed over the skin
surface area. Of these patients, 61% had generalized
lymphadenopathy at the time of the first examination. Four of these patients,
who had generalized lymphadenopathy in the absence of skin lesions or
detectable visceral organ involvement at the time of presentation, were found to
have biopsy-proven KS localized to the lymph nodes. In 45% of the patients
studied, KS lesions were found in one or more sites along the gastrointestinal
tract. Of these patients, 29% had either unexplained fever or
unexplained weight loss when first seen. While most patients present with skin
disease, KS involvement of lymph nodes or the gastrointestinal tract may
occasionally precede the appearance of the cutaneous lesions.
Eventually, most patients with epidemic KS develop disseminated disease. The disease often progresses in an orderly fashion from a few localized or
widespread mucocutaneous lesions to more numerous lesions and generalized skin disease
with lymph node, gastrointestinal tract disease, and other organ involvement.
Pleuropulmonary KS is an ominous sign usually occurring late in the course of
the disease, especially in those patients whose death is directly attributed to
KS. Most patients with epidemic KS die of one or more complicating
Several reports documented KS in homosexual men who
persistently had no evidence of HIV infection. These patients had an
indolent and cutaneous form of the disease, which caused new lesions to appear every
few years. Lesions occur most commonly on the extremities and genitalia but
can occur anywhere on the skin. These cases may indicate the presence of
causal factors other than HIV that homosexual men may be exposed to because of
Friedman-Kien AE, Saltzman BR, Cao YZ, et al.: Kaposi's sarcoma in HIV-negative homosexual men. Lancet 335 (8682): 168-9, 1990.
Safai B: Kaposi's sarcoma and acquired immunodeficiency syndrome. In: DeVita VT, Hellman S, Rosenberg S, eds.: AIDS: Etiology, Diagnosis, Treatment and Prevention. 4th ed. Philadelphia, Pa: Lippincott-Raven Publishers, 1997, pp 295-318.
Chang Y, Cesarman E, Pessin MS, et al.: Identification of herpesvirus-like DNA sequences in AIDS-associated Kaposi's sarcoma. Science 266 (5192): 1865-9, 1994.
Moore PS, Chang Y: Detection of herpesvirus-like DNA sequences in Kaposi's sarcoma in patients with and without HIV infection. N Engl J Med 332 (18): 1181-5, 1995.
Su IJ, Hsu YS, Chang YC, et al.: Herpesvirus-like DNA sequence in Kaposi's sarcoma from AIDS and non-AIDS patients in Taiwan. Lancet 345 (8951): 722-3, 1995.
Gao SJ, Kingsley L, Li M, et al.: KSHV antibodies among Americans, Italians and Ugandans with and without Kaposi's sarcoma. Nat Med 2 (8): 925-8, 1996.
Chang Y, Ziegler J, Wabinga H, et al.: Kaposi's sarcoma-associated herpesvirus and Kaposi's sarcoma in Africa. Uganda Kaposi's Sarcoma Study Group. Arch Intern Med 156 (2): 202-4, 1996.
Safai B, Good RA: Kaposi's sarcoma: a review and recent developments. Clin Bull 10 (2): 62-9, 1980.
Reynolds WA, Winkelmann RK, Soule EH: Kaposi's sarcoma: a clinicopathologic study with particular reference to its relationship to the reticuloendothelial system. Medicine (Baltimore) 44 (5): 419-43, 1965.
Safai B, Miké V, Giraldo G, et al.: Association of Kaposi's sarcoma with second primary malignancies: possible etiopathogenic implications. Cancer 45 (6): 1472-9, 1980.
Taylor JF, Templeton AC, Vogel CL, et al.: Kaposi's sarcoma in Uganda: a clinico-pathological study. Int J Cancer 8 (1): 122-35, 1971.
Templeton AC, Bhana D: Prognosis in Kaposi's sarcoma. J Natl Cancer Inst 55 (6): 1301-4, 1975.
Penn I: Kaposi's sarcoma in organ transplant recipients: report of 20 cases. Transplantation 27 (1): 8-11, 1979.
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Vogel J, Hinrichs SH, Reynolds RK, et al.: The HIV tat gene induces dermal lesions resembling Kaposi's sarcoma in transgenic mice. Nature 335 (6191): 606-11, 1988.
Selik RM, Starcher ET, Curran JW: Opportunistic diseases reported in AIDS patients: frequencies, associations, and trends. AIDS 1 (3): 175-82, 1987.
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Palella FJ Jr, Delaney KM, Moorman AC, et al.: Declining morbidity and mortality among patients with advanced human immunodeficiency virus infection. HIV Outpatient Study Investigators. N Engl J Med 338 (13): 853-60, 1998.
Lodi S, Guiguet M, Costagliola D, et al.: Kaposi sarcoma incidence and survival among HIV-infected homosexual men after HIV seroconversion. J Natl Cancer Inst 102 (11): 784-92, 2010.
Portsmouth S, Stebbing J, Gill J, et al.: A comparison of regimens based on non-nucleoside reverse transcriptase inhibitors or protease inhibitors in preventing Kaposi's sarcoma. AIDS 17 (11): F17-22, 2003.
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Dupont C, Vasseur E, Beauchet A, et al.: Long-term efficacy on Kaposi's sarcoma of highly active antiretroviral therapy in a cohort of HIV-positive patients. CISIH 92. Centre d'information et de soins de l'immunodéficience humaine. AIDS 14 (8): 987-93, 2000.
Tam HK, Zhang ZF, Jacobson LP, et al.: Effect of highly active antiretroviral therapy on survival among HIV-infected men with Kaposi sarcoma or non-Hodgkin lymphoma. Int J Cancer 98 (6): 916-22, 2002.
Carrieri MP, Pradier C, Piselli P, et al.: Reduced incidence of Kaposi's sarcoma and of systemic non-hodgkin's lymphoma in HIV-infected individuals treated with highly active antiretroviral therapy. Int J Cancer 103 (1): 142-4, 2003.
Grabar S, Abraham B, Mahamat A, et al.: Differential impact of combination antiretroviral therapy in preventing Kaposi's sarcoma with and without visceral involvement. J Clin Oncol 24 (21): 3408-14, 2006.
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The staging evaluation of patients with classic Kaposi sarcoma (KS) should be
individualized. The advanced age of most of the patients, localized nature
of the tumor, rarity of visceral involvement, and usually indolent course of
the disease should temper the extent of the evaluation. A careful examination
of the skin and lymph nodes is sufficient in most cases. For the rare
patient with rapidly progressive tumor or signs or symptoms of visceral
involvement, appropriate evaluation is indicated. No universally
accepted classification is available for epidemic KS. Staging schemes that incorporate laboratory parameters as well as clinical features have been proposed. Since
most patients with epidemic KS do not die from the disease, factors besides tumor burden are apparently involved in survival.
The conventions used to stage KS and the methods used to evaluate the benefits
of KS treatment continue to evolve because of changes in
the treatment of human immunodeficiency virus (HIV) and in recognition of deficiencies in standard tumor
assessment. The clinical course of KS, the selection of treatment, and
the response to treatment are heavily influenced by the degree of underlying immune
dysfunction and opportunistic infections.
The AIDS Clinical Trials Group (ACTG) Oncology Committee has published criteria
for the evaluation of epidemic KS. The staging system incorporates
measures of extent of disease, severity of immunodeficiency, and presence of
systemic symptoms. As shown in Table 1 below, the ACTG criteria categorizes the extent of
the tumor as localized or disseminated, the CD4 cell number as high or low, and
a systemic illness as absent or present.
A subsequent prospective analysis of
294 patients entered on ACTG trials for KS between 1989 and 1995 showed that
each of the tumor, immune system, and systemic illness variables was
independently associated with survival. Multivariate analysis showed that
immune system impairment was the most important single predictor of survival.
In patients with relatively high CD4 counts, tumor stage was predictive. A CD4
count of 150 cells/mm³ may be a better discriminator than the
published cutoff of 200 cells/mm³. A study is in progress to
determine if viral load adds predictive information. None of the prior studies were conducted at a time when highly active
antiretroviral therapy (HAART) was readily available. The impact of HAART on
survival in KS requires continued assessment.
Good Risk (0)
Poor Risk (1)
(Any of the following)
Confined to skin
and/or lymph nodes
and/or minimal oral
diseaseMinimal oral disease is non-nodular KS confined to the palate.
Tumor-associated edema or ulceration
Extensive oral KS
KS in other non-nodal
Immune system (I)
CD4 cells ≥ = 200/microL
CD4 cells <200 per
Systemic illness (S)
No history of OIs or
thrushOIs are opportunistic infections.
History of OIs and/or thrush
No “B” symptoms “B” symptoms are unexplained fever, night sweats, >10%
involuntary weight loss, or diarrhea persisting >2 weeks.
“B” symptoms present
Krown SE, Metroka C, Wernz JC: Kaposi's sarcoma in the acquired immune deficiency syndrome: a proposal for uniform evaluation, response, and staging criteria. AIDS Clinical Trials Group Oncology Committee. J Clin Oncol 7 (9): 1201-7, 1989.
Krown SE, Testa MA, Huang J: AIDS-related Kaposi's sarcoma: prospective validation of the AIDS Clinical Trials Group staging classification. AIDS Clinical Trials Group Oncology Committee. J Clin Oncol 15 (9): 3085-92, 1997.
Classic Kaposi sarcoma (KS) usually is limited to the skin and has an indolent
course. Patients with this tumor are predisposed to the development of a
second primary malignancy, and the treating physician should consider this
factor when arranging a schedule of follow-up treatment for the patient.
Equivalent standard treatment options:
Widespread skin disease:
EBRT used in this manner gave long-term results that were superior to
those obtained with radiation therapy administered to successive individual lesions as they
One patient was treated repeatedly with intralesional injections of 0.25
to 0.50 mg of vincristine, which resulted in complete disappearance of the treated lesion.
Multiple courses of therapy were required because of the recurrence of disease in
Lymph node and gastrointestinal tract involvement:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with classic Kaposi sarcoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Hamilton CR, Cummings BJ, Harwood AR: Radiotherapy of Kaposi's sarcoma. Int J Radiat Oncol Biol Phys 12 (11): 1931-5, 1986.
Nisce LZ, Safai B, Poussin-Rosillo H: Once weekly total and subtotal skin electron beam therapy for Kaposi's sarcoma. Cancer 47 (4): 640-4, 1981.
Solan AJ, Greenwald ES, Silvay O: Long-term complete remissions of Kaposi's sarcoma with vinblastine therapy. Cancer 47 (4): 637-9, 1981.
Tucker SB, Winkelmann RK: Treatment of Kaposi sarcoma with vinblastine. Arch Dermatol 112 (7): 958-61, 1976.
Scott WP, Voight JA: Kaposi's sarcoma. Management with vincaleucoblastine. Cancer 19 (4): 557-64, 1966.
Klein E, Schwartz RA, Laor Y, et al.: Treatment of Kaposi's sarcoma with vinblastine. Cancer 45 (3): 427-31, 1980.
Di Lorenzo G, Kreuter A, Di Trolio R, et al.: Activity and safety of pegylated liposomal doxorubicin as first-line therapy in the treatment of non-visceral classic Kaposi's sarcoma: a multicenter study. J Invest Dermatol 128 (6): 1578-80, 2008.
Odom RB, Goette DK: Treatment of cutaneous Kaposi's sarcoma with intralesional vincristine. Arch Dermatol 114 (11): 1693-4, 1978.
Some patients with Kaposi Sarcoma (KS) have noted spontaneous and lasting
remissions following discontinuation of immunosuppressive therapy. In managing these patients, if immunosuppressive therapy is not critical, its discontinuation is a reasonable first step.
Standard treatment options:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with immunosuppressive treatment related Kaposi sarcoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
Cohen L: Dose, time, and volume parameters in irradiation therapy of Kaposi's sarcoma. Br J Radiol 35 (415): 485-488, 1962.
Lo TC, Salzman FA, Smedal MI, et al.: Radiotherapy for Kaposi's sarcoma. Cancer 45 (4): 684-7, 1980.
Treatment may result:
No data are available, however, to show that treatment improves
survival. In addition to antitumor treatment, essential components of an
optimal Kaposi sarcoma (KS) treatment strategy include highly active antiretroviral
treatment (HAART), prophylaxis for opportunistic infections, and rapid recognition and
treatment of intercurrent infections.
Most good-risk patients, as defined by the AIDS Clinical Trials Group, show tumor regression with HAART alone. Poor-risk patients usually require a combination of HAART and chemotherapy with discontinuation of the chemotherapy after disappearance of the skin lesion.
Small localized lesions of KS may be treated by electrodesiccation and
curettage, cryotherapy, or by surgical excision. KS tumors are also generally
very responsive to local radiation therapy, and excellent palliation has been
obtained with doses at 20 Gy or slightly higher. One report
demonstrated a response rate higher than 90%, with a median time to progression
of 21 months. Although no difference in response was noted with a variety of
fractionation regimens, a single fraction of 8 Gy is indicated for cutaneous
lesions and is associated with significantly fewer severe reactions.
Radiation therapy is generally reserved to treat localized areas of the skin
and oral cavity. It is less often used to control pulmonary, gastrointestinal
tract, or other sites of KS lesions. Localized KS lesions have also
been effectively treated with intralesional injections of vinblastine.
Alitretinoin 0.1% gel provided local control in a randomized prospective multicenter trial.[Level of evidence: 1iiDiv]
In epidemic KS, the already profoundly depressed immunologic status of the host
limits the therapeutic usefulness of systemic chemotherapy. Systemic
chemotherapy studies in epidemic KS have used as single agents or in
combinations doxorubicin, bleomycin, vinblastine, vincristine, etoposide, paclitaxel, and docetaxel.[Level of evidence: 3iiiDiv]
Randomized multicenter trials
showed an improvement in response rate (45%–60% vs. 20%–25%) and a more favorable toxic effects
profile for pegylated liposomal doxorubicin or liposomal daunorubicin, compared to the combination of doxorubicin, bleomycin, and vincristine or bleomycin and vincristine.[Level of evidence: 1iiDiv]
During HAART, both pegylated liposomal doxorubicin and paclitaxel are active single agents with response rates close to 50%.[Level of evidence: 1iiDiv]
The interferon alphas have also been widely studied and show a 40% objective
response rate in patients with epidemic KS. In these reports, the
responses differed significantly according to the prognostic factors of extent
of disease, prior or coexistent opportunistic infections, prior treatment with
chemotherapy, CD4 lymphocyte counts lower than 200 cells/mm³,
the presence of circulating acid-labile interferon alpha, and an increase in
beta-2-microglobulin. Several treatment studies have combined interferon alpha
with other chemotherapeutic agents. Overall, these trials have shown no
benefit with the interferon-chemotherapy combinations as compared to the
Recombinant interferon alpha-2a and interferon alpha-2b were the first agents
approved for the treatment of KS. Approval was based on single-agent studies
performed in the 1980s before the advent of antiretroviral therapy. The early
studies demonstrated improved efficacy at relatively high doses. High-dose
monotherapy is rarely used today, and instead, interferon is given in
combination with other anti-HIV drugs in doses of 4 to 18 million units.
Neutropenia is dose limiting, and trials of doses of 1 to 10 million units
combined with less myelosuppressive antiretrovirals are in progress. Response
to interferon is slow, and the maximum effect is seen after 6 or more months.
Interferon should probably not be used in the treatment of patients with rapidly progressive,
Bevacizumab, the humanized, antivascular, endothelial growth–factor monoclonal antibody, had a response rate in 5 of 16 patients who did not improve after the institution of HAART and chemotherapy.[Level of evidence: 3iiiDiv]
Interleukin-12 had a response rate of 71% (95% confidence interval, 48%–89%) among 24 evaluable patients in a phase I and phase II trial.[Level of evidence: 3iiiDiv]
Treatment options under clinical evaluation:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with AIDS-related Kaposi sarcoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
Krown SE: Highly active antiretroviral therapy in AIDS-associated Kaposi's sarcoma: implications for the design of therapeutic trials in patients with advanced, symptomatic Kaposi's sarcoma. J Clin Oncol 22 (3): 399-402, 2004.
Cooper JS, Steinfeld AD, Lerch I: Intentions and outcomes in the radiotherapeutic management of epidemic Kaposi's sarcoma. Int J Radiat Oncol Biol Phys 20 (3): 419-22, 1991.
Nobler MP, Leddy ME, Huh SH: The impact of palliative irradiation on the management of patients with acquired immune deficiency syndrome. J Clin Oncol 5 (1): 107-12, 1987.
Singh NB, Lakier RH, Donde B: Hypofractionated radiation therapy in the treatment of epidemic Kaposi sarcoma--a prospective randomized trial. Radiother Oncol 88 (2): 211-6, 2008.
Berson AM, Quivey JM, Harris JW, et al.: Radiation therapy for AIDS-related Kaposi's Sarcoma. Int J Radiat Oncol Biol Phys 19 (3): 569-75, 1990.
Epstein JB, Lozada-Nur F, McLeod WA, et al.: Oral Kaposi's sarcoma in acquired immunodeficiency syndrome. Review of management and report of the efficacy of intralesional vinblastine. Cancer 64 (12): 2424-30, 1989.
Bodsworth NJ, Bloch M, Bower M, et al.: Phase III vehicle-controlled, multi-centered study of topical alitretinoin gel 0.1% in cutaneous AIDS-related Kaposi's sarcoma. Am J Clin Dermatol 2 (2): 77-87, 2001.
Evans SR, Krown SE, Testa MA, et al.: Phase II evaluation of low-dose oral etoposide for the treatment of relapsed or progressive AIDS-related Kaposi's sarcoma: an AIDS Clinical Trials Group clinical study. J Clin Oncol 20 (15): 3236-41, 2002.
Saville MW, Lietzau J, Pluda JM, et al.: Treatment of HIV-associated Kaposi's sarcoma with paclitaxel. Lancet 346 (8966): 26-8, 1995.
Lim ST, Tupule A, Espina BM, et al.: Weekly docetaxel is safe and effective in the treatment of advanced-stage acquired immunodeficiency syndrome-related Kaposi sarcoma. Cancer 103 (2): 417-21, 2005.
Gill PS, Tulpule A, Espina BM, et al.: Paclitaxel is safe and effective in the treatment of advanced AIDS-related Kaposi's sarcoma. J Clin Oncol 17 (6): 1876-83, 1999.
Di Lorenzo G, Konstantinopoulos PA, Pantanowitz L, et al.: Management of AIDS-related Kaposi's sarcoma. Lancet Oncol 8 (2): 167-76, 2007.
Stewart S, Jablonowski H, Goebel FD, et al.: Randomized comparative trial of pegylated liposomal doxorubicin versus bleomycin and vincristine in the treatment of AIDS-related Kaposi's sarcoma. International Pegylated Liposomal Doxorubicin Study Group. J Clin Oncol 16 (2): 683-91, 1998.
Northfelt DW, Dezube BJ, Thommes JA, et al.: Pegylated-liposomal doxorubicin versus doxorubicin, bleomycin, and vincristine in the treatment of AIDS-related Kaposi's sarcoma: results of a randomized phase III clinical trial. J Clin Oncol 16 (7): 2445-51, 1998.
Gill PS, Wernz J, Scadden DT, et al.: Randomized phase III trial of liposomal daunorubicin versus doxorubicin, bleomycin, and vincristine in AIDS-related Kaposi's sarcoma. J Clin Oncol 14 (8): 2353-64, 1996.
Cianfrocca M, Lee S, Von Roenn J, et al.: Randomized trial of paclitaxel versus pegylated liposomal doxorubicin for advanced human immunodeficiency virus-associated Kaposi sarcoma: evidence of symptom palliation from chemotherapy. Cancer 116 (16): 3969-77, 2010.
Real FX, Oettgen HF, Krown SE: Kaposi's sarcoma and the acquired immunodeficiency syndrome: treatment with high and low doses of recombinant leukocyte A interferon. J Clin Oncol 4 (4): 544-51, 1986.
Groopman JE, Gottlieb MS, Goodman J, et al.: Recombinant alpha-2 interferon therapy for Kaposi's sarcoma associated with the acquired immunodeficiency syndrome. Ann Intern Med 100 (5): 671-6, 1984.
Uldrick TS, Wyvill KM, Kumar P, et al.: Phase II study of bevacizumab in patients with HIV-associated Kaposi's sarcoma receiving antiretroviral therapy. J Clin Oncol 30 (13): 1476-83, 2012.
Little RF, Pluda JM, Wyvill KM, et al.: Activity of subcutaneous interleukin-12 in AIDS-related Kaposi sarcoma. Blood 107 (12): 4650-7, 2006.
The prognosis for any treated Kaposi sarcoma patient with progressing,
recurring, or relapsing disease is highly variable. Deciding on further
treatment depends on many factors, most importantly the clinical setting
(i.e., classic, immunosuppressive treatment, or AIDS) in which the tumor arises as
well as individual patient considerations.
Clinical trials are appropriate and
should be considered when possible.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent Kaposi sarcoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
This information was last updated on June 26, 2013.
Our licensed social workers are here to help adult patients and their loved ones face the many new concerns and anxieties following a cancer diagnosis, offering emotional support and assistance with obtaining needed resources.
If you are dealing with the death of a loved one, grief can be a lonely and isolating experience. The Bereavement Program provides support to bereaved family members and friends following the death of a patient.
Concierge Services is your one-stop place to learn about Dana-Farber programs, services and resources, as well as information on getting around Boston, finding lodging or restaurants, and activities in the area.
The Expressive Arts Therapy program, sponsored by the Leonard P. Zakim Center for Integrative Therapies, provides adult patients, family members, and caregivers with a variety of options to support well-being during cancer treatment. From live music meditation to painting technique workshops, the program offers a range of creative outlets to suit every interest.
Dana-Farber and Brigham and Women's Hospital, including parking facilities, are fully accessible to people with disabilities. There are wheelchairs at the main entrance, and security staff can provide personal assistance. We also have many educational materials available in large print and audiotape formats.
The Ethics Consultation Service is available for patients and families who may be facing difficult decisions and choices regarding care. Our goal is to bring together patients, families and health care providers to talk about ethical concerns and help everyone involved arrive at a resolution that is right for all.
This comprehensive resource offers guidance, information and resources to support the entire family, including how to talk to children about cancer, advice for the well partner, and creating a support network.
Find practical tips and suggestions for individuals caring for a family member or friend with cancer, including creating a caregiving plan, finding community resources, and looking after your own well-being.
Friends' Place provides personal consultations to help cancer patients of all ages cope with changes in physical appearance that result from cancer treatment. Our experienced, compassionate team provides fittings for compression garments or breast prostheses, helps with wigs and other head coverings, and offers make-up and skincare advice.
The Friends' Corner Gift Shop, located on the first floor of the Yawkey Center for Cancer Care, offers a wide selection of unique gifts and everyday items for patients, families and staff.
Dana-Farber offers several services to help you and your family manage the financial side of cancer treatment. From creating bill payment schedules and estate planning advice to debt management and resource assistance for patients in need, our team is here for you.
Every year, thousands of patients with cancer from around the world come to Dana-Farber for their care. We provide a wide array of logistical and other services for individuals who live outside the United States.
Dana-Farber provides interpreting services for patients whose first language is not English. Interpreters may be requested for any activity, including registration, booking appointments, attending treatments and exams, support groups, and meetings with doctors and other members of your health care team.
Our nutritionists are registered dietitians who can assist you in planning an optimal diet during any stage of your cancer journey, cope with any side effects you may experience, and answer your questions about the latest findings on cancer and nutrition.
One-to-One connects adult patients, family members and caregivers with individuals who have gone through cancer themselves, providing an experienced and reassuring perspective for those facing a cancer diagnosis, treatment and recovery.
The Eleanor and Maxwell Blum Patient and Family Resource Center and its satellite resource rooms are staffed by health care professionals and provide computer stations, books, brochures, videos, and CDs to help you find information and support on a variety of issues about cancer treatment and care.
Patients websites help friends and family members stay up-to-date on their loved ones' condition and write messages of support and encouragement.
The Dana-Farber pharmacy fills prescriptions for all pediatric and adult patients. Our pharmacists are an extension of the patient care team and work closely with your physicians to provide seamless, convenient, safe care.
More than 1,200 Dana-Farber patients and their families have enjoyed free trips to baseball games, theater shows, museums, and other attractions this year through the Recreational Resources program.
The Sexual Health Program provides education, consultation and personalized rehabilitation for patients and their partners who have experienced changes in sexual health during and after cancer treatment.
Through all stages of cancer treatment and survivorship, our Spiritual Care staff is available 24 hours a day to provide emotional and spiritual support for adults and pediatric patients and family members.
Young adults with cancer face very different challenges than patients who were diagnosed earlier in childhood or later in adulthood. The Young Adult Program can help you to find the resources and expertise available at Dana-Farber to help support your cancer experience.
Integrative therapies, also known as complementary therapies, range from acupuncture and massage to nutritional guidance and music therapy. Patients treated at the Zakim Center credit its services with easing nausea, improving circulation, and reducing pain, stress, and anxiety associated with cancer treatment.
In this video, Dr. George Demetri talks about his work in the Sarcoma and Bone Cancer Treatment Center at Dana-Farber/Brigham and Women's Cancer Center