Sarcoma, Kaposi

  • Dana-Farber/Brigham and Women's Cancer Care

    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.

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: 

  • Alveolar soft part sarcoma
  • Angiosarcoma
  • Chondrosarcoma
  • Desmoid tumor
  • Desmoplastic small cell tumor
  • Epithelioid sarcoma
  • Ewings sarcoma
  • Extraskeletal mesenchymal chondrosarcoma
  • Extraskeletal osteosarcoma
  • Fibrous histiocytoma of bone
  • Fibrosarcoma
  • Gastrointestinal stromal tumor (GIST)
  • Kaposi's sarcoma
  • Leiomyosarcoma
  • Liposarcoma
  • Malignant fibrous histiocytoma (MFH)
  • Malignant mesenchymoma
  • Malignant primative neuroectodermal tumor (PNET)
  • Myofibroblastic sarcoma
  • Myxofibrosarcoma
  • Neurofibrosarcoma
  • Osteoganic sarcoma
  • Osteosarcoma
  • PEComa
  • Rhabdomyosarcoma
  • Malignant schwannoma
  • Spindle cell sarcoma
  • Synovial sarcoma

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General Information About Kaposi Sarcoma

Kaposi sarcoma is a disease in which malignant tumors (cancer) can form in the skin, mucous membranes, lymph nodes, and other organs.

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:

  • Classic Kaposi sarcoma.
  • African Kaposi sarcoma.
  • Immunosuppressive therapy–related Kaposi sarcoma.
  • Epidemic Kaposi sarcoma.
  • Nonepidemic Kaposi sarcoma.

Tests that examine the skin, lungs, and gastrointestinal tract are used to detect (find) and diagnose Kaposi sarcoma.

The following tests and procedures may be used:

  • Physical exam and history: An exam of the body to check general signs of health, including checking skin and lymph nodes for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.
  • Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer.
  • Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. This is used to find Kaposi sarcoma in the lungs.
  • Endoscopy: A procedure to look at organs and tissues inside the body to check for abnormal areas. An endoscope is inserted through an incision (cut) in the skin or opening in the body, such as the mouth. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease. This is used to find Kaposi sarcoma lesions in the gastrointestinal tract.
  • Bronchoscopy: A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

  • The type of Kaposi sarcoma.
  • The general health of the patient, especially the patient's immune system.
  • Whether the cancer has just been diagnosed or has recurred (come back).

Classic Kaposi Sarcoma

Classic Kaposi sarcoma is found most often in older men of Italian or Eastern European Jewish origin.

Classic Kaposi sarcoma is a rare disease that gets worse slowly over many years.

Symptoms of classic Kaposi sarcoma may include slow-growing lesions on the legs and feet.

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.

Another cancer may develop.

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

African Kaposi sarcoma is a fairly common form of the disease found in young adult males who live near the equator in Africa. Symptoms 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

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

Epidemic Kaposi sarcoma is found in patients who have acquired immunodeficiency syndrome (AIDS).

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.

Symptoms of epidemic Kaposi sarcoma can include lesions that form in many parts of the body.

The symptoms of epidemic Kaposi sarcoma can include lesions in different parts of the body, including any of the following:

  • Skin.
  • Lining of the mouth.
  • Lymph nodes.
  • Stomach and intestines.
  • Lungs and lining of the chest.
  • Liver.
  • Spleen.

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.

The use of drug therapy called HAART reduces the risk of epidemic Kaposi sarcoma in patients infected with HIV.

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.

Nonepidemic Gay-related Kaposi Sarcoma

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.

Recurrent Kaposi Sarcoma

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.

Treatment Option Overview

There are different types of treatment for patients with Kaposi sarcoma.

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.

Treatment of epidemic Kaposi sarcoma combines treatment for Kaposi sarcoma with treatment for AIDS.

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.

Four types of standard treatment are used to treat Kaposi sarcoma:

Radiation therapy

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.

Surgery

The following surgical procedures may be used for Kaposi sarcoma to treat small, surface lesions:

  • Local excision: The cancer is cut from the skin along with a small amount of normal tissue around it.
  • Electrodesiccation and curettage: The tumor is cut from the skin with a curette (a sharp, spoon-shaped tool). A needle-shaped electrode is then used to treat the area with an electric current that stops the bleeding and destroys cancer cells that remain around the edge of the wound. The process may be repeated one to three times during the surgery to remove all of the cancer.
  • Cryosurgery: A treatment that uses an instrument to freeze and destroy abnormal tissue. This type of treatment is also called cryotherapy.

Chemotherapy

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

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.

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI Web site.

Targeted therapy

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.

Patients may want to think about taking part in a clinical trial.

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.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

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.

Follow-up tests may be needed.

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 Options for Kaposi Sarcoma

Classic Kaposi Sarcoma

Treatment for single lesions may include the following:

  • Radiation therapy.
  • Surgery.

Treatment for lesions all over the body may include the following:

  • Radiation therapy.
  • Chemotherapy.

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.

Immunosuppressive Therapy–related Kaposi Sarcoma

Treatment for immunosuppressive therapy–related Kaposi sarcoma may include the following:

  • Stopping or reducing immunosuppressive drugtherapy.
  • Radiation therapy.
  • Chemotherapy using one or more anticancer drugs.

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.

Epidemic Kaposi Sarcoma

Treatment for epidemic Kaposi sarcoma may include the following:

  • Surgery, including local excision or electrodesiccation and curettage.
  • Cryosurgery.
  • Radiation therapy.
  • Chemotherapy using one or more anticancer drugs.
  • Biologic therapy.
  • A clinical trial of new drug therapy, biologic therapy, or targeted therapy.

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.

Recurrent Kaposi Sarcoma

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.

To Learn More About Kaposi Sarcoma

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 September 19, 2013.


General Information About Kaposi Sarcoma

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.[1]

Although 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.[2] 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.[3] 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 tissue.[4][5][6][7]

Classic Kaposi Sarcoma

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.[8][9][10]

African Kaposi Sarcoma

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.[11][12]

Immunosuppressive Treatment–Related Kaposi Sarcoma

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.[13] 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.

Epidemic Kaposi Sarcoma

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.[14] 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.[15]

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.[16] 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.[17][18][19] The use of HAART has been associated with a sustained and substantial decline in KS incidence in multiple large cohorts.[20][21][22][23][24][25]

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.[26] 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.[27] Most patients with epidemic KS die of one or more complicating opportunistic infections.

Nonepidemic Gay–Related Kaposi Sarcoma

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.[1] These cases may indicate the presence of causal factors other than HIV that homosexual men may be exposed to because of their lifestyle.

References:

  1. Friedman-Kien AE, Saltzman BR, Cao YZ, et al.: Kaposi's sarcoma in HIV-negative homosexual men. Lancet 335 (8682): 168-9, 1990.

  2. 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.

  3. 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.

  4. 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.

  5. 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.

  6. 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.

  7. 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.

  8. Safai B, Good RA: Kaposi's sarcoma: a review and recent developments. Clin Bull 10 (2): 62-9, 1980.

  9. 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.

  10. 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.

  11. 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.

  12. Templeton AC, Bhana D: Prognosis in Kaposi's sarcoma. J Natl Cancer Inst 55 (6): 1301-4, 1975.

  13. Penn I: Kaposi's sarcoma in organ transplant recipients: report of 20 cases. Transplantation 27 (1): 8-11, 1979.

  14. Kaposi's sarcoma and Pneumocystis pneumonia among homosexual men--New York City and California. MMWR Morb Mortal Wkly Rep 30 (25): 305-8, 1981.

  15. 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.

  16. Selik RM, Starcher ET, Curran JW: Opportunistic diseases reported in AIDS patients: frequencies, associations, and trends. AIDS 1 (3): 175-82, 1987.

  17. Flexner C: HIV-protease inhibitors. N Engl J Med 338 (18): 1281-92, 1998.

  18. 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.

  19. 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.

  20. 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.

  21. International Collaboration on HIV and Cancer.: Highly active antiretroviral therapy and incidence of cancer in human immunodeficiency virus-infected adults. J Natl Cancer Inst 92 (22): 1823-30, 2000.

  22. 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.

  23. 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.

  24. 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.

  25. 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.

  26. Krigel RL, Laubenstein LJ, Muggia FM: Kaposi's sarcoma: a new staging classification. Cancer Treat Rep 67 (6): 531-4, 1983.

  27. Gill PS, Akil B, Colletti P, et al.: Pulmonary Kaposi's sarcoma: clinical findings and results of therapy. Am J Med 87 (1): 57-61, 1989.

Stage Information for Kaposi Sarcoma

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.[1] 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.[2] 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.

Table 1. AIDS Clinical Trials Group Staging Classification

Good Risk (0)

Poor Risk (1)

(Any of the following)

(Any of the following)

Tumor (T)

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

Gastrointestinal KS

KS in other non-nodal viscera

Immune system (I)

CD4 cells ≥ = 200/microL

CD4 cells <200 per cubic millimeter

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

Performance status ≥70 (Karnofsky)

Performance status <70

Other HIV-related illness (e.g., neurological disease or lymphoma)

References:

  1. 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.

  2. 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 Treatment

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:

Solitary lesions:

  1. Radiation therapy: For solitary lesions or lesions of limited extent, modest doses of radiation applied to the lesions with a limited margin provide excellent control of disease in the treated area. Usually, superficial radiation beams such as electron beams are used. Some authors believe disease recurrence in adjacent, untreated skin is common if only involved-field radiation therapy is used and claim better cure rates when extended-field radiation therapy is used.[1][2]
    • Low-voltage (100 kv) photon radiation: 8 Gy to 10 Gy given as a single dose or 15 Gy to 20 Gy given over 1 week because solitary lesions control nearly 100% of local disease, but recurrence in adjacent areas is common.
    • Electron-beam radiation therapy (EBRT): 4 Gy given once weekly for 6 to 8 consecutive weeks with a 4-MeV to 6-MeV electron beam. Ports should include the entire skin surface 15 cm above the lesion.
  2. Surgical excision may be of benefit in some patients with small superficial lesions, but local recurrence is likely to be a problem. However, over the years, multiple small excisions can be performed to achieve good disease control.

Widespread skin disease:

  1. Radiation therapy: Modest doses are effective in controlling disease. The type of radiation (i.e., photon vs. electron) and fields used must be tailored to suit the distribution of disease in the individual patient.[2]
    • Extended-field EBRT.
    • For disease limited to areas distal to the knee, subtotal-skin EBRT directed to skin below the umbilicus.
    • For disease that extends above the knee, total-skin EBRT.

    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 appeared.[2]

    • EBRT: 4 Gy given once weekly for 6 to 8 consecutive weeks, and subtotal- or total-skin radiation therapy given for extensive disease.
  2. Chemotherapy: Because classic KS is such a rare disease in the United States and is usually treated initially with radiation therapy, few patients have been treated with chemotherapy, and no randomized prospective trials have compared one agent to another. Several authors have used single-agent vinblastine given as a weekly dose of approximately 0.1 mg/kg.[3][4][5][6] Almost all of the patients had good to excellent response. In most cases, patients required prolonged courses of therapy, for several years, to maintain a partial response. Doses of vinblastine were titrated in individual patients to maintain a white blood count of more than 3,000 leukocytes. Follow-up after completion of therapy was not presented. In a multicenter trial of 55 patients who were treated over a decade, a 71% overall response rate was seen using pegylated liposomal doxorubicin.[7][Level of evidence: 3iiiDiv]

    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.[8] Multiple courses of therapy were required because of the recurrence of disease in untreated areas.

Lymph node and gastrointestinal tract involvement:

  1. Chemotherapy: Several patients who had widespread skin disease and were treated with chemotherapy also had lymph node and gastrointestinal tract involvement. The disease in these sites also responded to vinblastine. Trials are required to define therapy. One such trial, MSKCC-04055, has been completed.
  2. Local radiation therapy may be added to chemotherapy if individual lesions require urgent therapy.

Current Clinical Trials

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.

References:

  1. Hamilton CR, Cummings BJ, Harwood AR: Radiotherapy of Kaposi's sarcoma. Int J Radiat Oncol Biol Phys 12 (11): 1931-5, 1986.

  2. 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.

  3. Solan AJ, Greenwald ES, Silvay O: Long-term complete remissions of Kaposi's sarcoma with vinblastine therapy. Cancer 47 (4): 637-9, 1981.

  4. Tucker SB, Winkelmann RK: Treatment of Kaposi sarcoma with vinblastine. Arch Dermatol 112 (7): 958-61, 1976.

  5. Scott WP, Voight JA: Kaposi's sarcoma. Management with vincaleucoblastine. Cancer 19 (4): 557-64, 1966.

  6. Klein E, Schwartz RA, Laor Y, et al.: Treatment of Kaposi's sarcoma with vinblastine. Cancer 45 (3): 427-31, 1980.

  7. 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.

  8. Odom RB, Goette DK: Treatment of cutaneous Kaposi's sarcoma with intralesional vincristine. Arch Dermatol 114 (11): 1693-4, 1978.

Immunosuppressive Therapy–Related Kaposi Sarcoma Treatment

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:

  1. Discontinue immunosuppressive therapy (often results in tumor regression). This option is critically important in patients who are receiving immunosuppressive drugs, as in the case of certain transplant patients.
  2. Radiation therapy (for disease limited to skin).[1][2][3][4]
  3. Chemotherapy (single or multiple drug): Most systemic chemotherapy trials in KS patients have been carried out in the African and epidemic varieties. See the section on the treatment of Epidemic Kaposi Sarcoma. The applicability of the results of these trials to KS in immunosuppressed patients is unknown.

Current Clinical Trials

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.

General information about clinical trials is also available from the NCI Web site.

References:

  1. Cohen L: Dose, time, and volume parameters in irradiation therapy of Kaposi's sarcoma. Br J Radiol 35 (415): 485-488, 1962.

  2. Hamilton CR, Cummings BJ, Harwood AR: Radiotherapy of Kaposi's sarcoma. Int J Radiat Oncol Biol Phys 12 (11): 1931-5, 1986.

  3. Lo TC, Salzman FA, Smedal MI, et al.: Radiotherapy for Kaposi's sarcoma. Cancer 45 (4): 684-7, 1980.

  4. 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.

Epidemic Kaposi Sarcoma Treatment

Treatment may result:

  1. In a disappearance or reduction in size of specific skin lesions, thereby alleviating the discomfort associated with the chronic edema and ulcerations that often accompany multiple skin tumors seen on the lower extremities.
  2. In control of symptoms associated with mucosal or visceral lesions.

No data are available, however, to show that treatment improves survival.[1] 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.[2] Poor-risk patients usually require a combination of HAART and chemotherapy with discontinuation of the chemotherapy after disappearance of the skin lesion.[2]

Local modalities

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.[3][4][5] 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.[6] 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.[7] Alitretinoin 0.1% gel provided local control in a randomized prospective multicenter trial.[8][Level of evidence: 1iiDiv]

Chemotherapy

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.[9][10][11][12][13][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.[14][15][16][Level of evidence: 1iiDiv] During HAART, both pegylated liposomal doxorubicin and paclitaxel are active single agents with response rates close to 50%.[17][Level of evidence: 1iiDiv]

Biologic therapy

The interferon alphas have also been widely studied and show a 40% objective response rate in patients with epidemic KS.[18][19] 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 single-agent activities.

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, symptomatic KS.

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.[20][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.[21][Level of evidence: 3iiiDiv]

Treatment options under clinical evaluation:

  • Patients with epidemic KS are appropriate candidates for clinical trials evaluating new drugs or biologicals.

Current Clinical Trials

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.

General information about clinical trials is also available from the NCI Web site.

References:

  1. 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.

  2. 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.

  3. 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.

  4. 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.

  5. 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.

  6. 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.

  7. 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.

  8. 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.

  9. 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.

  10. Saville MW, Lietzau J, Pluda JM, et al.: Treatment of HIV-associated Kaposi's sarcoma with paclitaxel. Lancet 346 (8966): 26-8, 1995.

  11. 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.

  12. 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.

  13. Di Lorenzo G, Konstantinopoulos PA, Pantanowitz L, et al.: Management of AIDS-related Kaposi's sarcoma. Lancet Oncol 8 (2): 167-76, 2007.

  14. 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.

  15. 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.

  16. 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.

  17. 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.

  18. 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.

  19. 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.

  20. 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.

  21. Little RF, Pluda JM, Wyvill KM, et al.: Activity of subcutaneous interleukin-12 in AIDS-related Kaposi sarcoma. Blood 107 (12): 4650-7, 2006.

Recurrent Kaposi Sarcoma Treatment

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.

Current Clinical Trials

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.

General information about clinical trials is also available from the NCI Web site.


This information is provided by the National Cancer Institute.

This information was last updated on June 26, 2013.

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