Table of Contents Purpose of This PDQ Summary General Information About Kaposi Sarcoma
Stage Information for Kaposi Sarcoma Classic Kaposi Sarcoma Immunosuppressive Treatment–Related Kaposi Sarcoma Epidemic Kaposi Sarcoma Recurrent Kaposi Sarcoma Get More Information From NCI Changes to This Summary (05/16/2008) More Information
Purpose of This PDQ Summary
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of Kaposi sarcoma. This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board.
Information about the following is included in this summary:
- Epidemiology and clinical presentation.
- Cellular classification.
- Staging.
- Treatment options for different types of tumor.
This summary is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.
Some of the reference citations in the summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations. Based on the strength of the available evidence, treatment options are described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for reimbursement determinations.
This summary is available in a patient version, written in less technical language, and in Spanish.
Back to Top 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.[4-7]
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.[8-10]
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] The use of HAART has been associated with a sustained and substantial decline in KS incidence in multiple large cohorts.[19-24]
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.[25]
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.[26] 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
-
Friedman-Kien AE, Saltzman BR, Cao YZ, et al.: Kaposi's sarcoma in HIV-negative homosexual men. Lancet 335 (8682): 168-9, 1990.
[PUBMED Abstract]
-
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.
[PUBMED Abstract]
-
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.
[PUBMED Abstract]
-
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.
[PUBMED Abstract]
-
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.
[PUBMED Abstract]
-
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.
[PUBMED Abstract]
-
Safai B, Good RA: Kaposi's sarcoma: a review and recent developments. Clin Bull 10 (2): 62-9, 1980.
[PUBMED Abstract]
-
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.
[PUBMED Abstract]
-
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.
[PUBMED Abstract]
-
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.
[PUBMED Abstract]
-
Templeton AC, Bhana D: Prognosis in Kaposi's sarcoma. J Natl Cancer Inst 55 (6): 1301-4, 1975.
[PUBMED Abstract]
-
Penn I: Kaposi's sarcoma in organ transplant recipients: report of 20 cases. Transplantation 27 (1): 8-11, 1979.
[PUBMED Abstract]
-
Kaposi's sarcoma and Pneumocystis pneumonia among homosexual men--New York City and California. MMWR Morb Mortal Wkly Rep 30 (25): 305-8, 1981.
[PUBMED Abstract]
-
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.
[PUBMED Abstract]
-
Selik RM, Starcher ET, Curran JW: Opportunistic diseases reported in AIDS patients: frequencies, associations, and trends. AIDS 1 (3): 175-82, 1987.
[PUBMED Abstract]
-
Flexner C: HIV-protease inhibitors. N Engl J Med 338 (18): 1281-92, 1998.
[PUBMED Abstract]
-
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.
[PUBMED Abstract]
-
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.
[PUBMED Abstract]
-
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.
[PUBMED Abstract]
-
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.
[PUBMED Abstract]
-
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.
[PUBMED Abstract]
-
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.
[PUBMED Abstract]
-
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.
[PUBMED Abstract]
-
Krigel RL, Laubenstein LJ, Muggia FM: Kaposi's sarcoma: a new staging classification. Cancer Treat Rep 67 (6): 531-4, 1983.
[PUBMED Abstract]
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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.
[PUBMED Abstract]
Back to Top 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 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 the table 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 (TIS) 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.
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
disease [Note: Minimal 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
thrush [Note: OIs are opportunistic infections.]
|
History of OIs and/or thrush |
No “B” symptoms [Note: “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
-
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.
[PUBMED Abstract]
-
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.
[PUBMED Abstract]
Back to Top Classic Kaposi Sarcoma
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:
- 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 to 10 Gy given as a single dose
or 15 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.
- 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:
- 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.
- 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.[3-6]
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.
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.[7]
Multiple courses of therapy were required because of the recurrence of disease in
untreated areas.
Lymph node and gastrointestinal tract involvement:
- 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. No studies are otherwise available to address the treatment of
visceral classic KS specifically.
- 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 PDQ Cancer Clinical Trials Registry 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
-
Hamilton CR, Cummings BJ, Harwood AR: Radiotherapy of Kaposi's sarcoma. Int J Radiat Oncol Biol Phys 12 (11): 1931-5, 1986.
[PUBMED Abstract]
-
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.
[PUBMED Abstract]
-
Solan AJ, Greenwald ES, Silvay O: Long-term complete remissions of Kaposi's sarcoma with vinblastine therapy. Cancer 47 (4): 637-9, 1981.
[PUBMED Abstract]
-
Tucker SB, Winkelmann RK: Treatment of Kaposi sarcoma with vinblastine. Arch Dermatol 112 (7): 958-61, 1976.
[PUBMED Abstract]
-
Scott WP, Voight JA: Kaposi's sarcoma. Management with vincaleucoblastine. Cancer 19 (4): 557-64, 1966.
[PUBMED Abstract]
-
Klein E, Schwartz RA, Laor Y, et al.: Treatment of Kaposi's sarcoma with vinblastine. Cancer 45 (3): 427-31, 1980.
[PUBMED Abstract]
-
Odom RB, Goette DK: Treatment of cutaneous Kaposi's sarcoma with intralesional vincristine. Arch Dermatol 114 (11): 1693-4, 1978.
[PUBMED Abstract]
Back to Top Immunosuppressive Treatment–Related Kaposi Sarcoma
Some patients with 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:
- 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.
- Radiation therapy (for disease limited to skin).[1-4]
- 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 PDQ Cancer Clinical Trials Registry 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
-
Cohen L: Dose, time, and volume parameters in irradiation therapy of Kaposi's sarcoma. Br J Radiol 35(415): 485-488, 1962.
-
Hamilton CR, Cummings BJ, Harwood AR: Radiotherapy of Kaposi's sarcoma. Int J Radiat Oncol Biol Phys 12 (11): 1931-5, 1986.
[PUBMED Abstract]
-
Lo TC, Salzman FA, Smedal MI, et al.: Radiotherapy for Kaposi's sarcoma. Cancer 45 (4): 684-7, 1980.
[PUBMED Abstract]
-
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.
[PUBMED Abstract]
Back to Top Epidemic Kaposi Sarcoma
Note: Some citations in the text of this section are followed by a level of
evidence. The PDQ editorial boards use a formal ranking system to help the
reader judge the strength of evidence linked to the reported results of a
therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence for more
information.)
Treatment may result:
- 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.
- 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 electrode siccation 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 not much larger than 20 Gy.[3,4] 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.[5]
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.[6]
Alitretinoin 0.1% gel provided local control in a randomized prospective multicenter trial.[7][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.[8-12][Level of evidence: 3iiiDiv]
Randomized multicenter trials
showed an improvement in response rate (45% to 60% vs. 20% to 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.[13-15][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.[16,17] 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.
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.[18][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 PDQ Cancer Clinical Trials Registry 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
-
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.
-
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.
[PUBMED Abstract]
-
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.
[PUBMED Abstract]
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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.
[PUBMED Abstract]
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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.
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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.
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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.
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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.
[PUBMED Abstract]
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Saville MW, Lietzau J, Pluda JM, et al.: Treatment of HIV-associated Kaposi's sarcoma with paclitaxel. Lancet 346 (8966): 26-8, 1995.
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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.
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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.
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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.
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Back to Top Recurrent Kaposi Sarcoma
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 PDQ Cancer Clinical Trials Registry 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.
Back to Top Get More Information From NCI
Call 1-800-4-CANCER
For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1-800-332-8615. The call is free and a trained Cancer Information Specialist is available to answer your questions.
Chat online
The NCI's LiveHelp® online chat service provides Internet users with the ability to chat online with an Information Specialist. The service is available from 9:00 a.m. to 11:00 p.m. Eastern time, Monday through Friday. Information Specialists can help Internet users find information on NCI Web sites and answer questions about cancer.
Write to us
For more information from the NCI, please write to this address:
- NCI Public Inquiries Office
- Suite 3036A
- 6116 Executive Boulevard, MSC8322
- Bethesda, MD 20892-8322
Search the NCI Web site
The NCI Web site provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. For a quick search, use our “Best Bets” search box in the upper right hand corner of each Web page. The results that are most closely related to your search term will be listed as Best Bets at the top of the list of search results.
There are also many other places to get materials and information about cancer treatment and services. Hospitals in your area may have information about local and regional agencies that have information on finances, getting to and from treatment, receiving care at home, and dealing with problems related to cancer treatment.
Find Publications
The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.
Back to Top Changes to This Summary (05/16/2008)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Editorial changes were made to this summary.
Back to Top More Information
About PDQ
Additional PDQ Summaries
Important:
This information is intended mainly for use by doctors and other health care professionals. If you have questions about this topic, you can ask your doctor, or call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).
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