Procedure
Note
Cytological diagnosis is usually not sufficient.
Mandatory
Procedure | Note |
---|---|
First choice method | |
First choice method | |
Supplementary in case of clinical or imaging suspicion of thoracic organ involvement | |
Supplementary in case of clinical or imaging suspicion of abdominal organ involvement | |
Supplementary in case of clinical or imaging suspicion of gastrointestinal involvement with therapeutic consequences | |
Supplementary in case of clinical or imaging suspicion of lung involvement with therapeutic consequences |
Sporadic, classic KS
KS associated with iatrogenic immunosuppression
Endemic, African KS
Epidemic, HIV-associated KS
KS in men who have sex with men (MSM) without HIV infection.
KS is a mesenchymal tumor of blood vessels and lymphatic vessels. The histologic image of KS is multifaceted and changes with clinical progression. KS consist of three components [ 8 ] :
Angiomatous phase
Spindle cell phase
Inflammatory phase
Closely adjacent to larger plexus vessels in the mid and upper stratum reticulare of the dermis, multicentric discrete perivascular spindle cell proliferations with slit-like clefts accompanied by lymphoplasmacytic infiltrates, extravascular erythrocytes, hemosiderin deposits, and siderophages ('pseudogranulomatous pattern') are found in early KS, initially omitting the papillary body and its vessels. In addition, endothelium-lined vascular clefts with empty lumina may dominate. Adnexa and preexisting vascular structures are partially encompassed by the newly formed vascular clefts and lacunae in a semi-island-like fashion ('promontory sign'). In early KS, mitoses and endothelial apoptosis are rare, and cellular and nuclear atypia are absent.
Spindle cells encroaching on the papillary body intersperse the entire corium bundled into short, cell-rich fascicles or strands. Sieve-like, the spindle cell aggregates are broken up by slit-like erythrocyte-rich clefts.
The tumor periphery is dominated by congested, dilated, serum-free vessels engorged with erythrocytes that appear stuffed ('stuffing'). Spindle cell apoptosis but no significant nuclear atypia is observed. Intracellularly and extracellularly, erythrocytic degradation is present in the form of hyaline PAS-positive globi ('hyaline globules').
Mitotic-rich, densely packed, factor XIIIa positive, CD31 positive, and CD34 positive, fascicular-structured spindle cell tumors with enclosed erythrocyte-rich clefts, moderate nuclear atypia (exceptionally marked atypia in anaplastic equatorial African variants) often surrounded by an epithelial collerette in exophytic growth, and by a connective tissue pseudocapsule in expansive nodular variants. PAS-positive hyaline erythrocytic globi and apoptotic spindle cells occur in clusters. Older lesions show necrosis in addition to hemorrhages and iron storage.
At regression, a plasma cell-rich, inflammatory round cell infiltrate develops.
Molecular detection of KS herpesvirus HHV-8 is helpful for differential diagnosis.
Prognostic factors are summarized in the TIS (Tumor, Immune system, Systemic Illness) classification of the ACTG (AIDS Clinical Trials Group) [ 15 ] , see Table 3 .
Criteria | |||
---|---|---|---|
Prognosis | Tumor characteristics | Immune system | Systemic involvement |
Skin/lymph node involvement only (minimal palate involvement) | No opportunistic infection, no B symptoms, and Karnofsky Performance Score ≥70% | ||
Tumor with edema or ulceration, extensive oral involvement, organ involvement other than lymph nodes | Opportunistic infections, other AIDS-defining diseases, B-symptomatic or Karnofsky PS <70% |
Differential diagnosis of KS should include acroangiodermatitis in chronic venous insufficiency, other angiosarcomas, hemangiomas, bacillary angiomatosis, Gougerot-Blum disease, melanoma metastases, and erythema elevatum et diutinum. In case of doubt, an excisional biopsy should be performed to confirm the histological diagnosis.
The treatment approach is based on the four epidemiologic clinical variants [ 1 , 17 ] . An overview of the primary therapeutic procedures is summarized in Figure 1 .
In classical KS, individualized treatment concepts and often only local therapy are preferred due to the typically higher age of the patients [ 22 ] . Since KS is radiosensitive, fractionated soft X-ray therapy, irradiation with fast electrons or cobalt irradiation are suitable [ 11 ] . Cryotherapies may also be used locally, as well as intralesionally applied vinca alkaloids, bleomycin, or interferons [ 4 , 20 ] . Excision should be considered only in cases of functionally compromising changes and rapid need for action. Systemic chemotherapy, usually with pegylated liposomal doxorubicin, is indicated for extensive primaries, systemic involvement, and progressive courses [ 12 , 23 ] .
The endemic African KS usually responds well to systemic therapy, with the exception of the lymphadenopathic variant.
In iatrogenic immunosuppression-associated KS, tumor lesions usually regress completely after cessation of immunosuppression.
In epidemic AIDS-associated KS, initiation of combined antiretroviral therapy (cART) in previously non-antiretrovirally treated patients often leads to an arrest of progression or even complete disappearance of the sarcoma lesions [ 7 ] . Therefore, antiretroviral therapy should be initiated at the latest with the occurrence of KS in HIV patients. The type of cART does not play a crucial role in KS response, but rather its virologic efficacy and cART-related immune reconstitution. In some cases, a transient, often substantial worsening of the condition may occur as part of the immune reconstitution syndrome. These patients should be treated additionally with systemic chemotherapy. Also, in cases of concurrent new diagnosis of advanced KS with organ involvement and HIV infection, concurrent initiation of antiretroviral and systemic KS therapy as described below is also recommended. If KS is diagnosed in patients already receiving antiretroviral therapy, its effectiveness should be reviewed and eventually be optimized using resistance testing.
Regardless of the clinical course, progressive KS should be treated systemically. In the past, a variety of substances were used for this purpose. These include interferon, vinca alkaloids, bleomycin and anthracyclines [ 9 ] .
Standard therapy for AIDS-associated or advanced KS consists of administration of pegylated liposomal doxorubicin at a dose of 20 mg per m² of body surface area at two to three weeks intervals until complete clinical remission. Generally, clinical follow-up is scheduled at 2-3 months. In case of good regression of tumor lesions without complete remission, further course is awaited after 4-6 months.
As with any initiation of anthracycline therapy, cardiac evaluation with echocardiography should be performed to determine left ventricular ejection fraction, as there is a risk of cardiotoxicity in addition to myelotoxicity. Painful macular erythema (palmoplantar erythrodysesthesia) may occur on the hands and feet associated with the administration of liposomal doxorubicin. However, this side effect is rarely observed at the doses recommended for KS, and more often at higher single doses.
The taxane paclitaxel is available for second-line therapy. In the original report by Gill et al., a dose of 100 mg/m² every two weeks was used [ 25 ] . However, since weekly administration at reduced doses has now been shown to be better tolerated and at least as effective in other diseases such as breast carcinoma, weekly administration may also be discussed in KS, see Onkopedia Female Breast Cancer . Myelotoxicity, alopecia, and onychodystrophy should also be noted. Interactions with cART should be considered in HIV-infected patients with KS.
In HIV-associated KS, systemic interferons can be used alternatively if CD4 cell counts are good (>350 cells/µl) or immune status is good. Pegylated interferons (weekly administration possible, better tolerated than classical interferons) are not approved for KS, but may be more effective and easier to administer, as shown by case reports in AIDS-associated, but also in classical Kaposi's sarcoma [ 16 ] .
For local compromising (feet, face) KS lesions, local surgery or local drug application are often sufficient. These are inexpensive and well tolerated. Recurrence in the scars is common after surgical excision. KS are radiosensitive, so radiotherapy may also be used, preferably fractionated single doses of soft X-rays [ 8 ] . Other local therapies range from camouflage to intralesional injection of vincristine and to experimental topical use of retinoids [ 3 ] .
After successful treatment of KS, esthetically disturbing postinflammatory hyperpigmentation often remains visible for a long time, which should not be confused with active KS.
8.1 [chapter not relevant].
Since KS is prone to recurrence, regular surveillance must be performed.
AWMF Guideline S1 Kaposi's sarcoma, 2021. https://register.awmf.org/de/leitlinien/detail/032-025 .
Brockmeyer NH, Mertins L. Therapy of HIV-associated Kaposi's sarcoma. IN: Brockmeyer NH, Mertins L (eds.): HIV infection, pathogenesis, diagnosis, therapy. Springer Verlag Berlin Heidelberg New York 1997. p. 93-122. ISBN 3-540-62544-5.
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 2:77-87, 2001. PMID:11705307
Boudreaux AA, Smith LL, Cosby CD et al. Intralesional vinblastine for cutaneous Kaposi`s sarcoma associated with acquired immunodeficiency syndrome. J Am Acad Dermatol 28:61-65. 1993. PMID:8381146
Cesarman E, Damania B, Krown SE et al. Kaposi sarcoma. Nat Rev Dis Primers 5:9, 2019. DOI:10.1038/s41572-019-0060-9
Denis D, Seta V, Regnier-Rosencher E et al. A fifth subtype of Kaposi's sarcoma, classic Kaposi's sarcoma in men who have sex with men: a cohort study in Paris. J Eur Acad Dermatol Venereol 32:1377-1384, 2018. DOI:10.1111/jdv.14831
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 14:987-993, 2000. PMID:10853980
Fritsch P, Zelger B, Sepp N. Kaposi's sarcoma. Histology. IN: Fritsch P (ed.) Dermatology and venereology. Springer Verlag. Berlin, Heidelberg, New York. p. 617. ISBN 3-540-61169-X.
Gill PS, Wenz J, Scadden DT et al. Randomized phase III trial of daunorubicin versus doxorubicin, bleomycin, and vincristine in AIDS-related Kaposi’s sarcoma. J Clin Oncol 8:2353-2364, 1996. DOI:10.1200/JCO.1996.14.8.2353
Guo LN, Nambudiri VE. Kaposi sarcoma in HIV-negative men who have sex with men: a case series of nonepidemic Kaposi sarcoma. Clin Exp Dermatol 585-587, 2020. DOI:10.1111/ced.14146
Hamilton CR, Cummings BJ, Harwood AR. Radiotherapy of Kaposi's sarcoma. Int J Radiat Oncol Biol Phys 12:1931-1935, 1986. PMID:3771313
Hengge UR, Esser S, Rudel HP, Goos M. Long-term chemotherapy of HIV-associated Kaposi's sarcoma with liposomal doxorubicin. Eur J Cancer 37:878-883, 2001. PMID:11313176
Hensel M, Goetzenich A, Lutz T et al. HIV and cancer in Germany. Dtsch Arztebl Int 108:117-122, 2011. DOI:10.3238/arztebl.2010.0117
Kaposi M. Idiopathic multiple pigmentary sarcoma of the skin. Arch Derm Syph 4:265-273, 1872.
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 7:1201-1207, 1989. DOI:10.1200/JCO.1989.7.9.1201
Krown SE, Li P, Von Roenn JH et al. Efficacy of low-dose interferon with antiretroviral therapy in Kaposi`s sarcoma: a randomized phase II AIDS clinical trials group study. J Interferon Cytokine Res 22:295-303, 2002. DOI:10.1089/107999002753675712
Lanternier F, Lebbe C, Schartz N et al. Kaposi's sarcoma in HIV-negative men having sex with men. AIDS 22:1163-1168, 2008. DOI:10.1097/QAD.0b013e3283031a8a
Lebbe C, Garbe C, Stratigos AJ et al. Diagnosis and treatment of Kaposi's sarcoma: European consensus-based interdisciplinary guideline (EDF/EADO/EORTC). Eur J Cancer 114:117-127, 2019. DOI:10.1016/j.ejca.2018.12.036
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 332:1181-1185, 1995. DOI:10.1056/NEJM1995043321801
Myskowski PL. Intralesional interferon a-2b produces responses in Kaposi’s sarcoma. Dermatology 3:11, 1992.
Rashidghamat E, Bunker CB, Bower M, Banerjee P. Kaposi sarcoma in HIV-negative men who have sex with men. Br J Dermatol 171:1267-1268, 2014. DOI:10.1111/bjd.13102
Régnier-Rosencher E, Guillot B, Dupin N. Treatments for classic Kaposi sarcoma: a systematic review of the literature. J Am Acad Dermatol 68: 313-331, 2013. DOI:10.1016/j.jaad.2012.04.018
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: 683-691, 1998. DOI:10.1200/JCO.1998.16.2.683
Stürzl M, Blasig C, Schreier A et al. Expression of HHV-8 latency-associated T0.7 RNA in spindle cells and endothelial cells of AIDS-associated, classical and African Kaposi's sarcoma. Int J Cancer 72:68-71, 1997 PMID:9212225
Tulpule A, Groopman J, Saville MW et al. Multicenter trial of low-dose paclitaxel in patients with advanced AIDS-related Kaposi sarcoma. Cancer 95:147-154, 2002. DOI:10.1002/cncr.10634
Yanik EL, Achenbach CJ, Gopal S et al. Changes in clinical context for Kaposi's Sarcoma and Non-Hodgkin Lymphoma among people with HIV infection in the United States J Clin Oncol 34: 3276-3283, 2016. DOI:10.1200/JCO.2016.67.6999
11 systemic therapy - protocols, 12 study results, 13 certification status.
Author | Employer | Consulting / Expert opinion | Shares / Funds | Patent / Copyright / License | Fees | Funding of scientific research | Other financial relations | Personal relationship with authorized representatives |
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Mosthaf, Franz A. | ||||||||
Esser, Stefan |
Quellenangabe:
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General information about kaposi sarcoma, kaposi sarcoma is a disease in which malignant lesions (cancer) can form in the skin, mucous membranes, lymph nodes, and other organs., tests that examine the skin, lungs, and gastrointestinal tract are used to diagnose kaposi sarcoma., after kaposi sarcoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body., certain factors affect prognosis (chance of recovery) and treatment options..
Kaposi sarcoma is a cancer that causes lesions ( abnormal tissue ) 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 with HHV-8 do not get Kaposi sarcoma. People with HHV-8 are more likely to develop Kaposi sarcoma if their immune system is weakened by disease, such as human immunodeficiency virus (HIV), or by drugs given after an organ transplant .
There are several types of Kaposi sarcoma. The two types discussed in this summary include:
In addition to asking about your personal and family health history and doing a physical exam , your doctor may perform the following tests and procedures:
One of the following types of biopsies may be done to check for Kaposi sarcoma lesions in the skin:
An endoscopy or bronchoscopy may be done to check for Kaposi sarcoma lesions in the gastrointestinal tract or lungs.
The following tests and procedures may be used to find out if cancer has spread to other parts of the body:
The prognosis and treatment options depend on the following:
Classic kaposi sarcoma is found most often in older men of italian or eastern european jewish origin., signs of classic kaposi sarcoma may include slow-growing lesions on the legs and feet., another cancer may develop..
Classic Kaposi sarcoma is a rare disease that gets worse slowly over many years.
Patients may have one or more red, purple, or brown skin lesions on the legs and feet, most often on the ankles or soles of the feet. Over time, lesions may form in other parts of the body, such as the stomach , intestines , or lymph nodes . The lesions usually don't cause any symptoms but may grow in size and number over a period of 10 years or more. Pressure from the lesions may block the flow of lymph and blood in the legs and cause painful swelling. Lesions in the digestive tract may cause gastrointestinal bleeding.
Some patients with classic Kaposi sarcoma may develop another type of cancer before the Kaposi sarcoma lesions appear or later in life. Most often, this second cancer is non-Hodgkin lymphoma . Frequent follow-up is needed to watch for these second cancers.
Patients with hiv are at risk of developing epidemic kaposi sarcoma (hiv-associated kaposi sarcoma)., the use of drug therapy called highly active antiretroviral therapy (haart) reduces the risk of epidemic kaposi sarcoma in patients with hiv., signs of epidemic kaposi sarcoma can include lesions that form in many parts of the body..
AIDS is caused by HIV , which attacks and weakens the body's immune system . A weakened immune system is unable to fight infection and disease. People with HIV have an increased risk of infection and cancer .
A person with HIV and certain types of infection or cancer, such as Kaposi sarcoma , is diagnosed as having AIDS. Sometimes, a person is diagnosed with AIDS and epidemic Kaposi sarcoma at the same time.
HAART is a combination of several drugs used to lessen the damage to the immune system caused by HIV infection. Treatment with HAART reduces the risk of epidemic Kaposi sarcoma, although it is possible for a person to develop epidemic Kaposi sarcoma while taking HAART.
For information about AIDS and its treatment, see the HIVinfo website .
The signs of epidemic Kaposi sarcoma can include lesions in different parts of the body, including any of the following:
Kaposi sarcoma is sometimes found in the lining of the mouth during a regular dental check-up.
In most patients with epidemic Kaposi sarcoma, the disease will spread to other parts of the body over time.
There are different types of treatment for patients with kaposi sarcoma., radiation therapy, cryosurgery, chemotherapy, immunotherapy, targeted therapy, treatment for kaposi sarcoma may cause side effects., patients may want to think about taking part in a clinical trial., patients can enter clinical trials before, during, or after starting their cancer treatment., follow-up tests may be needed..
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 acquired immunodeficiency syndrome (AIDS). The types of treatment used to treat Kaposi sarcoma include:
Highly active antiretroviral therapy (HAART) is a combination of several drugs used to lessen the damage to the immune system caused by human immunodeficiency virus (HIV) infection. For many patients, HAART alone may be enough to treat epidemic Kaposi sarcoma. For other patients, HAART may be combined with other standard treatments to treat epidemic Kaposi sarcoma.
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:
The way the radiation therapy is given depends on the type of the cancer being treated. Certain types of external radiation therapy are used to treat Kaposi sarcoma lesions . Photon radiation therapy treats lesions with high-energy light. Electron beam radiation therapy uses tiny negatively charged particles called electrons .
The following surgical procedures may be used for Kaposi sarcoma to treat small, surface lesions:
Cryosurgery is a treatment that uses an instrument to freeze and destroy abnormal tissue. This type of treatment is also called cryotherapy.
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 , tissue, or a body cavity such as the abdomen , the drugs mainly affect cancer cells in those areas ( regional chemotherapy ).
In electrochemotherapy, intravenous chemotherapy is given and a probe is used to send electric pulses to the tumor. The pulses make an opening in the membrane around the tumor cell and allow the chemotherapy to get inside.
The way the chemotherapy is given depends on where the Kaposi sarcoma lesions occur in the body. In Kaposi sarcoma, chemotherapy may be given in the following ways:
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 Kaposi Sarcoma for more information.
Immunotherapy 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. Immunotherapy with interferon alfa and interleukin-12 may be used to treat Kaposi sarcoma.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website .
Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Monoclonal antibody therapy and tyrosine kinase inhibitors (TKIs) are types of targeted therapy being studied in the treatment of Kaposi sarcoma.
Monoclonal antibodies are immune system proteins made in the laboratory to treat many diseases, including cancer. As a cancer treatment, these antibodies can attach to a specific target on cancer cells or other cells that may help cancer cells grow. The antibodies are able to then kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion . They may be used alone or to carry drugs, toxins , or radioactive material directly to cancer cells. Bevacizumab is a monoclonal antibody that may be used to treat Kaposi sarcoma.
TKIs block signals needed for tumors to grow. Imatinib mesylate is a TKI that may be used to treat Kaposi sarcoma.
For information about side effects caused by treatment for cancer, visit our Side Effects page.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment .
Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
As you go through treatment, you will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated 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.
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).
For information about the treatments listed below, see the Treatment Option Overview section.
Treatment for single skin lesions may include the following:
Treatment for skin lesions all over the body may include the following:
Treatment for Kaposi sarcoma that affects lymph nodes or the gastrointestinal tract usually includes chemotherapy with or without radiation therapy.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Treatment for epidemic Kaposi sarcoma may include the following:
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, visit:
Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish .
PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.
This PDQ cancer information summary has current information about the treatment of Kaposi sarcoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Adult Treatment Editorial Board .
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." 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.
Clinical trials can be found online at NCI's website . For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
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The best way to cite this PDQ summary is:
PDQ® Adult Treatment Editorial Board. PDQ Kaposi Sarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/soft-tissue-sarcoma/patient/kaposi-treatment-pdq . Accessed <MM/DD/YYYY>. [PMID: 26389178]
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What you should be alert for in the history.
Kaposi’s sarcoma is characterized by the appearance of purplish, reddish-blue, or dark brown/black macules, plaques, or nodules on the skin. With classic Kaposi’s sarcoma, the lesions most commonly appear on the lower extremities (Figure 1).
Classical KS on the lower extremity. (Courtesy of Bryan Anderson, MD)
There are four types of Kaposi’s sarcoma:classic (sporadic), endemic (African), immunosuppression related and acquired immunodeficiency syndrome (AIDS) associated. In the cutaneous presentation is most often on the distal lower extremities the classic Kaposi’s and the immunosuppression related variety. In the endemic and AIDS-associated varieties, the disease is more often disseminated and aggressive, but invariably has cutaneous manifestations.
Visceral involvement, including the gastrointestinal tract and regional lymph nodes, is less common in the classic variety but more likely in the other subtypes. There are at least ten different morphologic variants of the cutaneous lesions of Kaposi’s sarcoma.
The skin lesions may appear as a plaque, patch, nodule, exophytic infiltrative, or lymphadenopathy. The lesions may appear cavernous and lymphangioma-like, and produce chronic lymphedema in the lower extremities. These lesions may consist of compressible nodules that appear as fluid-filled cysts, or they can coalesce and anastomose in networks, producing areas of extensive raised plaques.
Regional lymphadenopathy in the inguinal nodes may be present but does not influence prognosis or treatment options in patients with classic Kaposi’s.
Immunohistochemical staining of the biopsies can also be useful to detect the presence of LANA-1 (latency-associated nuclear antigen) within the spindle cells (Figure 2, Figure 3).
Histology of KS (H&E). Low power view. (Courtesy of Bryan Anderson, MD)
Histology of KS (H&E). High power view. (Courtesy of Bryan Anderson, MD)
Radiological studies are rarely necessary to evaluate potential metastatic disease sites in patients with classic Kaposi’s. Gastrointestinal involvement rarely complicates cases of classic Kaposi’s, except those with extensive skin involvement of the lower extremities. Patients with Kaposi’s related to endemic, immunosuppression, and AIDS are much more likely to have extensive disease involvement that is extracutaneous.
Involvement of the oral cavity occurs in approximately a third of patients with AIDS-related Kaposi’s, and in 15% of cases, it is the initial presentation of this disease. The palate is the most commonly affected area, followed by the gingiva. Intraoral lesions can be easily traumatized, causing pain, bleeding, ulceration, and secondary infection. Oral lesions may interfere with nutrition and speech, and are often a major reason for treating patients urgently.
Evaluation of gastrointestinal involvement should be performed by endoscopic examination, as these lesions are rarely seen radiologically. Gastrointestinal lesions may be asymptomatic, or can cause weight loss, abdominal pain, nausea, vomiting, upper or lower gastrointestinal bleeding, malabsorption, intestinal obstruction, or diarrhea.
Pulmonary involvement is common in AIDS-related Kaposi’s, usually presenting as shortness of breath, fever, cough, hemoptysis, or chest pain (or patients may be asymptomatic).
Radiological findings are variable, and include infiltrates and densities, effusions, and adenopathy. Diagnosis of pulmonary involvement with Kaposi’s sarcoma is usually made by bronchoscopy, by observing cherry-red raised lesions on the endobronchial surface.
Since a number of other organs can be involved by Kaposi’s computed tomography (CT) and magnetic resonance imaging (MRI) scans are usually needed to evaluate organ involvement including involvement of the lymph nodes, liver, pancreas, cardiovascular system, , testes, bone marrow, bone, or skeletal muscle.
Staging and prognosis are established by determining CD4 count, human immunodeficiency virus (HIV) viral load, and the presence of human herpes virus 8 (HHV-8) DNA. The most commonly used staging system developed by the AIDS clinical trial group separates patients into good and poor risk categories according to extent of tumor, immune status, and severity of illness. These factors were much more important in the period before highly active retroviral therapy. Since the introduction of highly active retroviral therapy, the incidence of and prognosis for Kaposi’s have improved substantially in all risk categories.
The diagnosis of classic Kaposi’s sarcoma is suspected based on the appearance and location of the lesions. Lesions located on the lower extremities can be mistaken for changes due to peripheral vascular disease or venous stasis.
Bacillary angiomatosis is a rare infection caused by the Bartonella species, which are gram-negative bacilli that are readily treated with antibiotics. Lesions due to this infection usually present as numerous small red-purple papules that may expand into larger exophytic lesions and become friable. The rash may be associated with fevers, chills, malaise, headache, and anorexia. Bacillary angiomatosis can infect both immunocompromised and immunocompetent patients.
Bacillary angiomatosis was described in the 1980s and found to be associated with AIDS at presentation. This condition may simulate Kaposi’s in patients with AIDS by its dermatological presentation and systemic spread; however, this disorder responds to antibiotics, including oral erythromycin as well as other antibiotics. Anti-tuberculosis medications are also effective. Bacillary angiomatosis is treatable and curable, but it may also be life-threatening, if undiagnosed.
Other rare infections that may produce similar and confusing nodular violaceous skin lesions include S. schenckii (sporotrichosis) and M. marium. Biopsy of these skin lesions allows a definitive diagnosis to be made. Biopsies demonstrate typical histological features under standard microscopy. Additional studies, including polymerase chain reaction, can be performed on obvious skin lesions to detect amplified HHV-8 DNA sequences.
There are four subgroups of Kaposi’s sarcoma:
1. Classic Kaposi’s is characteristically seen in older men of Mediterranean and Jewish origin.
2. Endemic or African Kaposi’s is found in all parts of equatorial Africa, particularly in sub-Saharan Africa, and is not associated with immunodeficiency.
HHV-8, also known as Kaposi’s-sarcoma-associated herpes virus (KSHV), is necessary but not sufficient for the development of Kaposi’s sarcoma. A secondary factor results in a reactivation of this virus, which is important in the pathogenesis of Kaposi’s and the distinctive and largely unexplained geographic distributions of classic Kaposi’s.
3. Immunosuppression due to transplantation, particularly solid organ transplant, is a risk factor for Kaposi’s sarcoma. Patients receiving immunosuppressive agents are not at risk for developing Kaposi’s; thus it appears that the immunological derangements that occur in patients with solid organ transplants and in those on immunosuppressive agents is a distinctive risk factor for Kaposi’s.
4. AIDS-related Kaposi’s (epidemic KS) is associated with HIV and severe immunosuppression with low CD4 counts. Kaposi’s sarcoma in patients with HIV defines AIDS. Treatment with highly active retroviral therapy not only helps control Kaposi’s disease, but also has been associated with a marked reduction in the incidence of Kaposi’s in patients on highly active retroviral therapy.
Kaposi’s sarcoma is a low-grade vascular tumor associated with infection with HHV-8 also known as KSHV. These vascular tumors primarily develop in the skin and lower extremities in classic Kaposi’s sarcoma and remain confined to the lower extremities, causing lymphedema and disfigurement.
Classic Kaposi’s patients are not immunosuppressed, and their disease involves chronic lower extremity edema due to this invasive tumor. This tumor rarely metastasizes distantly, other than to local regional nodes.
Endemic Kaposi’s follows a similar pattern, with lower extremity involvement, but generally is more aggressive and can disseminate to distant organs, unlike classic Kaposi’s.
Immunosuppression-related Kaposi’s occurs during immunosuppression for solid tumor organs. Lesions usually present in the skin and lower extremity, but are much more likely to disseminate. The control and progression of the lesions, both locally and with dissemination, are related to the ability to restore the immune system by eliminating or changing immunosuppressive agents.
AIDS-related Kaposi’s is a much more aggressive and widely disseminated disease, causing significant morbidity and mortality. The degree of immunosuppression is directly correlated with the risk of developing Kaposi’s sarcoma and treatment with highly active retroviral therapy; the resultant restoration of the immune system has reduced the incidence of Kaposi’s and provided an improved outcome.
Patients with AIDS-associated Kaposi’s or immunosuppression-mediated Kaposi’s who are treated with corticosteroids are more likely to have exacerbation of existing Kaposi’s lesions and progression of their lesions. Patients with Kaposi’s who are on corticosteroids also have a higher incidence of opportunistic infections.
Patients with AIDS-associated Kaposi’s that arises during immunosuppression are also at risk for other malignancies. Non-Hodgkin’s lymphoma is a common second malignancy in patients on immunosuppression. Non-Hodgkin’s lymphoma is a complication for patients with HIV-AIDS and for solid organ transplant patients that are being treated with immunosuppressive agents.
The subtype of Kaposi’s sarcoma will dictate the extent of clinical workup. For example, patients with classic Kaposi’s do not require any extensive radiological surveillance. On the other hand, patients with AIDS-related Kaposi’s will need very careful attention to various organs, and will require radiological studies, especially an endoscopic evaluation including careful oral and dental evaluations.
Chest x-rays are useful for screening for pulmonary lesions; bronchoscopy should be reserved for patients with abnormal radiographs or respiratory symptoms. Visceral disease is much more likely in patients with low CD4 counts (CD4 counts of 200 or less are usually associated with a higher risk of developing Kaposi’s and also with more aggressive and extensive disease at the time of presentation).
Testing stool for occult blood is a good screen for gastrointestinal involvement and endoscopy should be reserved for patients who have positive occult blood or have gastrointestinal symptoms.
Treatment options will vary according to the subtype of Kaposi’s.
CLASSIC KAPOSI’S SARCOMA
-observation of asymptomatic lesions
-compression therapy
-topical allotretinoin cream
-topical imiquimod
-intralesional interferon
-surgical excision
-cryosurgery
-laser (pulsed-dye laser)
-systemic chemotherapy (peglyated doxorubicin)
AFRICAN KAPOSI’S SARCOMA
African Kaposi’s sarcoma can be treated with the same approach used for patients with classic Kaposi’s.
IMMUNOSUPPRESSION-MEDIATED KAPOSI’S
-withdrawal of immunosuppressive agents
AIDS-ASSOCIATED KAPOSI’S SARCOMA
-highly active antiretroviral treatment
-intralesional chemotherapy (vincristine or bleomycin)
-laser ablation—pulsed dye laser
-systemic chemotherapy (liposomal anthracycline)
AIDS-associated Kaposi’s sarcoma patients who receive highly active retroviral therapy and develop immune reconstitution inflammatory syndrome and progressive Kaposi’s within weeks after initiation of highly active retroviral therapy should be treated with chemotherapy (vincristine, bleomycin, adriamycin, or paclitaxel based) during this phase.
Classic Kaposi’s sarcoma can be treated with surgical excision of small isolated lesions. If there is extensive skin involvement, then radiation therapy is the treatment of choice.
The goal of therapy is to alleviate symptoms, including reducing lymphedema, improving the function of lower extremities, decreasing the size of cutaneous lesions, and preventing progression. Patients with limited disease involvement may be observed without active therapy. Asymptomatic patients and those who do not have impairment of ambulation (if lower extremity edema can be managed with compressive stockings) may be observed without active therapy.
Patients with a limited volume of disease causing bleeding or cosmetic disfigurement can be treated with local therapy; radiation therapy, excision, cryotherapy, and laser ablation are useful, depending on the number of sites and the extent of involvement, as well as clinician and patient preference.
Patients with bulky disease that cannot be managed with local modalities should be considered for systemic therapy and first-line preference given to pegylated liposomal doxorubicin. African Kaposi’s can be treated with the same approach used for patients with classic Kaposi’s.
The recommendation is to initially diagnose the suspicious skin lesion and classify the subtype of Kaposi’s sarcoma. As indicated above, the various subtypes of Kaposi’s sarcoma will have different natural histories of disease. Once a diagnosis has been established, the patient can be referred to a medical oncologist and infectious disease specialist. For patients with a solid organ transplant who are on immunosuppression, the treatment decisions are usually difficult and require a balance of maintaining immunosuppression for the transplanted organ and controlling and eradicating the Kaposi’s sarcoma.
Immunosuppression-mediated Kaposi’s can often respond to withdrawal of the immunosuppressive agents used to control graft-versus-host disease. These maneuvers can often result in complete responses and remissions. Case reports have described regression of Kaposi’s sarcoma in patients treated with the mTOR inhibitor rapamycin (sirolimus), suggesting that this approach (rather than using an alternative immunosuppressive agent in transplant-associated Kaposi’s) may be much more rational and applicable to transplant patients.
Systemic chemotherapy with pegylated liposomal doxorubicin as a first-line therapy, followed by other second-line agents, can be used to control the disease in patients where immunosuppression cannot be altered.
Patients with AIDS-associated Kaposi’s who develop either limited or advanced Kaposi’s sarcoma should initially be treated with highly active retroviral therapy. For patients who have limited disease-causing symptoms or cosmetic disfigurement, local therapy rather than systemic therapy, in addition to highly active retroviral therapy, is the recommended approach. The treatment approach for local regional disease, including intralesional chemotherapy, surgery, cryotherapy, and laser ablation should be reserved for patients with small lesions.
Patients with larger lesions or bulky local regional disease should receive radiation therapy.
Patients with extensive cutaneous disease and symptomatic visceral involvement or local cutaneous disease unresponsive to local measures should receive systemic chemotherapy with the recommended liposomal anthracycline.
AIDS-associated Kaposi’s sarcoma patients who receive highly active retroviral therapy and develop immune reconstitution inflammatory syndrome and progressive Kaposi’s within weeks after initiation of highly active retroviral therapy should be treated with chemotherapy during this phase.
Classic Kaposi’s sarcoma requires the identification of the appropriate patient subgroup, namely older males of Mediterranean or Jewish ancestry. The lesions are typically extensive, involving the lower extremities, and treated with local regional control efforts. Laboratory studies need to be ordered to rule out HIV infection, and CD4 counts should be monitored. Radiological studies such as CT scans should be ordered only if there are symptoms and concern about organ involvement. There is no role for routine radiological surveillance.
African Kaposi’s sarcoma presents in a fashion similar to classic Kaposi’s, with lower extremity involvement; however, this disease tends to be more aggressive and infiltrative. Laboratory studies need to be ordered to rule out HIV infection, and CD4 counts should be monitored. Radiological studies, including CT scans and pulmonary and GI endoscopy, should be considered, especially in patients with symptoms and in those with extensive disease involvement.
AIDS-associated Kaposi’s sarcoma is a much more aggressive and widespread disease. Routine radiological surveillance and pulmonary and GI endoscopic studies should be performed. Physical examination should include a careful oral and dental evaluation. Laboratory studies include monitoring of viral load and CD4 counts.
Kaposi’s sarcoma that develops in patients with solid organ transplants is established once a positive skin biopsy is obtained. Radiological surveillance should include CT scans and endoscopic surveillance of the pulmonary system and GI tract. Laboratory studies will include monitoring of the immune system and viral serology, including HIV testing. Treatment decisions will be made by collaboration between the medical oncologist and transplant physician.
One must be vigilant for the possibility of secondary malignancies, most notably non-Hodgkin’s lymphoma.
Patients may be at risk for the immune reconstitution syndrome after the commencement of highly active antiretroviral therapy.
Gill, PS, Wernz, J, Scadden, DT. “Randomized phase 3 trial of liposomal doxorubicin in AIDS related Kaposi's sarcoma”. JClini Oncol. vol. 14. 1996. pp. 2353 (Nice study on the use of systemic chemotherapy for AIDS-related Kaposi's sarcoma)
Leidner, RS, Aboulafia, DM. “Recrudescent Kaposi's sarcoma after initiation of HAART: manifestation of immune reconstitution syndrome”. AIDS Patient Care STDS. vol. 19. 2005. pp. 635 (Kaposi's sarcoma manifests after the start of HAAART. Mechanisms and theories are discussed.)
Gallafent, JH, Buskin, SE, De Turk, PB, Aboulafia, DM. “Profile of patients with Kaposi's sarcoma in the era of highly active retroviral therapy”. JClini Oncol. vol. 23. 2005. pp. 1253 (Characterization of patients with Kaposi's, with special focus on those undergoing antiretroviral therapy)
Gill, PS, Loureiro, C, Bernstein-Singer, M. “Clinical effects of glucocorticoids on Kaposi's sarcoma related to acquired immunodeficiency syndrome”. Ann Intern Med. vol. 110. 1989. pp. 937 (Short paper on corticosteroids for the treatment of Kaposi's in patients with AIDS .)
Fenig, E, Brenner, B, Rakowsky, E. “Classic Kaposi's sarcoma: experience at Rabin Medical Center in Israel”. Am J Clin Oncol. vol. 21. 1998. pp. 498 (Wonderful review of the classical form of Kaposi's sarcoma. Discussion deals with etiology, pathogenesis, epidemiology, and therapy.)
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Paul curtiss.
The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, NY USA
Alvin e. friedman-kien.
Kaposi’s sarcoma is an angioproliferative neoplasm which has undergone considerable epidemiologic change since the original description by Moritz Kaposi in the late 1800s. This opportunistic neoplasm gained widespread notoriety within the US during the height of the AIDS epidemic, where it was frequently found co-occurring with opportunistic infections. With the advent of modern antiretroviral therapies, as well as an increasing number of individuals on immunosuppression for autoimmune disease or organ transplantation, the landscape of the immunocompromised individual has changed. It is now important for clinicians to be mindful of Kaposi’s sarcoma manifesting in a growing variety of clinical contexts.
Aids-associated kaposi’s sarcoma.
Kaposi’s sarcoma (KS) is a multi-focal, angioproliferative neoplasm that usually appears on the skin, but can also involve the visceral organs. In 1981, 26 cases of KS were reported as occurring in young homosexual men in New York and California. Many of these patients also had concomitant pneumocystis carinii pneumonia (PCP) and a variety of other “opportunistic infections” [ 1 ]. At that time, the underlying cause of AIDS was unknown, but, remarkably, one-third of these patients developed a disseminated form of KS. In 1983, it was postulated that KS was driven by the host’s profoundly immunocompromised state. Interestingly, 95% of these cases had occurred in homosexual men. Furthermore, it appeared that the clinical presentation of AIDS-associated KS (AIDS-KS) differed significantly from previously described clinical variants of this unusual neoplasm [ 2 ]. In these immunocompromised patients, KS behaved more aggressively, often involving mucosal tissues, and progressed to visceral involvement leading to organ dysfunction and death [ 3 ].
In the early 1980s, a significant proportion of homosexual men with AIDS were affected by KS; for example, in San Francisco, this opportunistic neoplasm occurred in 40% of homosexual men with AIDS. This suggested that a major risk factor for KS might be homosexual intercourse [ 4 ]. In fact, several early studies showed a connection between the number of sexual partners and the prevalence of KS. For example, in Vancouver, 56% of homosexual men with AIDS and more than 20 partners developed KS [ 5 ]. As the widespread use of highly active antiretroviral therapy (HAART) gained popularity in the early and mid-1990s, an 8.8% annual decline from 1990 to 1998 in KS incidence in the United States was observed, along with a 50% reduction in KS incidence among those on triple antiretroviral therapy [ 6 ]. A cancer surveillance program in San Francisco revealed similar patterns, with the incidence of KS in 1973 (prior to the AIDS epidemic) at 0.5 cases per 100,000 people, while during the peak of the AIDS epidemic in 1991, this number was 33.3 cases per 100,000 people and then declined in 1998 (post-HAART) to 2.8 cases per 100,000 people [ 7 ].
In 1872, Moritz Kaposi first described the “classic” variant of KS that is typically observed in elderly men of Mediterranean or eastern European origin [ 8 ]. This form of KS generally presents with lesions confined to the lower extremity, affects men more commonly than women in a 15:1 ratio, and is usually indolent with patients living 10 years or more [ 9 ]. The incidence of KS in the Mediterranean is significantly higher than in the rest of Europe and the United States. For example, prior to the AIDS epidemic, there was a two- to threefold higher prevalence of KS among the Italian population compared to that of the USA, and a ten times greater prevalence compared to England [ 10 ].
In the 1950s, an endemic form of KS was reported to be one of the most common neoplasms observed in central Africa, affecting men, women and children [ 11 ]. Notably, following the AIDS epidemic, the incidence of KS in Africa increased markedly, and from 1968 to 1970, KS accounted for 6.6% of all cancers occurring in men; however, from 1989 to 1991, KS became the most commonly reported cancer occurring in men (48.6% of male cancer patients), while the prevalence in female cancer patients rose to 17.9% [ 12 ].
In the 1970s, an “iatrogenic” form of KS was also observed among organ transplant recipients, as well as other patients on long-term immunosuppression for other diseases [ 2 ]. Recently, there have been a growing number of cases of this iatrogenic variant of KS. For example, there are an increasing number of reports in the literature describing individuals who develop KS arising in the context of long-term corticosteroid therapy and other biologic therapies, including rituximab, infliximab, and abatacept administered for chronic inflammatory and autoimmune conditions, including autoimmune thrombocytopenia, polyangiitis and pemphigus vulgaris [ 13 – 15 ]. The occurrence of KS in such patients frequently resolves spontaneously when the immunosuppressive therapies are discontinued [ 16 ]. As the increasing use of these immunomodulatory medications becomes more widespread, it is important to be aware of the possibility of KS occurring among these individuals who previously had not been considered “high-risk”.
New attention has been focused on the increased occurrence of KS among patients who are significantly immunosuppressed following organ transplantation. In fact, the incidence of KS is 400–500 times higher among such patients than the general population [ 17 ]. This may be due to reactivation of latent HHV-8 virus or perhaps through acquisition of the virus from the donor organ [ 18 ]. However, certain immunosuppressants may be less likely to put patients at risk for iatrogenic KS. For example, sirolimus or everolimus (mTOR inhibitors) appear to inhibit the occurrence and/or progression of KS in transplant recipients while preventing organ rejection [ 19 , 20 ]. These drugs may reduce the effect of vascular endothelial growth factor (VEGF) which is believed to play a pivotal role in the pathogenesis of KS [ 20 ]. Therefore, clinicians need to be aware of the oncogenic potential of immunosuppressive therapy in these organ transplant recipients.
For many years, the cause of KS was initially perplexing, with the occurrence of KS in patients such as those on long-term immunosuppressant therapy strongly suggesting the possibility that this neoplasm might be related to a transmissible agent. Interestingly, in the 1990s, there were also case reports of KS occurring in gay or bisexual HIV-negative immunocompetent individuals, supporting the hypothesis that KS was a neoplasm that could be due to a sexually transmitted infection occurring within that community [ 21 ]. It still remains unclear exactly why KS is almost exclusively observed in homosexual AIDS patients, and is very rarely if ever observed in IV drug users, or in individuals receiving blood products through transfusions, such as hemophiliacs. Perhaps the pathogenesis of KS may be hormone-dependent, which would account for the predominance in males and for the observation that KS is transferred between men through homosexual intercourse more commonly than from a male to a female partner through heterosexual intercourse [ 22 ].
In 1996, a newly discovered virus was found to be associated with every specimen of KS examined. Kaposi’s sarcoma-associated herpesvirus (KSHV), now known as human herpesvirus-8 (HHV8), was sequenced and identified as the infectious agent previously associated with KS among different populations, including individuals in Eastern Europe, Africa and the United States [ 23 ]. Since this discovery, new research has shed light on the molecular mechanisms of KS. The lesions found in KS are known to contain several cell types, such as infiltrating inflammatory cells, endothelium and spindle cells, which have been postulated to derive from either vascular or lymphatic precursors. One paper has shown a monoclonal antibody that stains specifically for lymphatic endothelium and also stains spindle cells from KS lesions, suggesting that the tumor cell of KS may be of lymphatic origin [ 24 ]. Moreover, spindle cell viability is likely dependent on the tumor milieu, which in KS consists of elevated levels of growth factors and cytokines, such as interleukin (IL)-1, IL-6, platelet-derived growth factor, tumor necrosis factor, IFN-gamma, and VEGF [ 25 ].
In HIV-infected individuals, Th1-type cytokines have been suggested to potentiate KSHV activation, which can lead to an increased viral load and a greater likelihood of the development of KS [ 26 ]. Also, HIV-1-associated trans-activating regulatory (Tat) protein is needed for replication of the HIV virus and is also released from acutely infected T cells. This protein may induce KS by stimulating proangiogenic chemokines, and by enhancing KS cell growth by synergizing with basic fibroblast growth factor (bFGF), which is an essential factor for lesion formation [ 3 ]. This may account for the more aggressive behavior of KS lesions in HIV-infected individuals compared to the classic variant [ 27 ]. However, the Tat protein is only able to interact with endothelial cells through a RGD motif, which is absent in HIV-2. This may explain why KS is less commonly found in individuals with HIV-2 compared to HIV-1 infection [ 28 ].
A variety of treatment options are available for the classic variant of KS. Since these lesions often recur, but are less often life-threatening, one viable treatment strategy is recurrent use of local cryotherapy which typically resolves the isolated lesions [ 29 ]. Another well-described approach is the use of intralesional chemotherapeutics such as vincristine which are also efficacious [ 30 ].
For patients with extensive disease and/or widespread rapidly progressive disease, it may be necessary to resort to more generalized therapies. Pegylated liposomal doxorubicin has been used as a first-line treatment, with a favorable 71% response rate, and is significantly better tolerated than traditional chemotherapeutics [ 31 , 32 ]. Another option is the use of localized radiation to the lower extremity which has shown a high rate of resolution to the affected fields [ 33 ].
Kaposi’s sarcoma occurring in patients on significant immunosuppression will resolve in the majority of patients when the immunosuppressive therapy is altered, reduced or discontinued. Commonly, when KS arises in renal transplant recipients, a decrease in the level of immunosuppression is a first reasonable strategy [ 34 ]. When this does not promote resolution of the disease, or begins to compromise graft viability, some patients may benefit from transition of typical calcineurin inhibitors such as cyclosporine to proliferation signal inhibitors such as sirolimus, which often results in complete resolution of active KS without rejection of the graft [ 20 ].
The mainstay of treatment for AIDS-related KS involves the initiation of HAART, which typically results in a subsequent decrease in tumor burden as the CD4 count rises and is able to adequately suppress the HHV8 virus. Interestingly, a subset of these patients may experience a progression of their disease following initiation of HAART in an immune reconstitution inflammatory syndrome (IRIS) [ 35 , 36 ]. Typically, these patients are able to continue therapy, but in some cases adjuvant chemotherapy may be required to control the tumor burden.
This article is based on previously conducted studies and does not involve any new studies of human or animal subjects performed by any of the authors.
No funding or sponsorship was received for this study or publication of this article. All named authors meet the International Committee of Medical Journal Editors (ICMJE) criteria for authorship for this manuscript, take responsibility for the integrity of the work as a whole, and have given final approval for the version to be published.
Paul Curtiss, Lauren C. Strazzulla and Alvin E. Friedman-Kien have nothing to disclose.
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Identify risk factors for Kaposi sarcoma. The clinician should ask about the following:
Demographics
Immune status
Previous skin lesions
Previous treatment for Kaposi sarcoma
History of opportunistic infections
Current medication use
Symptoms of Kaposi sarcoma include the following:
Photophobia
Recurrent red or bloody eyes
Irritation and foreign body sensation
Mucopurulent discharge
Heavy or swollen eyelids
Cosmetic disfigurement of the eyelids
Eyelashes rubbing against the eyes
Inability to close the eyes
Visual obstruction
Blurred vision
Full ocular examination should include the following:
Inspect and evert the eyelids and lashes
Perform slit lamp biomicroscopy
Examine palpebral and bulbar conjunctivae and fornices in detail
Palpate the lacrimal gland, and examine for masses
Examine both eyes for proptosis in the rare likelihood of orbital involvement; fortunately, intraocular Kaposi sarcoma has never been reported
The lesions are purplish-red to bright-red and highly vascular with surrounding telangiectatic vessels. They may be macular, plaquelike, or nodular. Ophthalmic Kaposi sarcoma lesions are found on the eyelids, conjunctiva, caruncle, and lacrimal sac. They rarely are found inside the orbit (1 case of choroidal involvement). [ 16 ]
See the image below.
Dugel et al described 3 clinical stages that may help direct therapy. Stage I and II tumors are patchy and flat. These lesions have a thickness of less than 3 mm in vertical height and are younger than 4 months. Stage III tumors are nodular and elevated with a vertical height of greater than 3 mm. They tend to be older than 4 months. [ 17 ]
Of ophthalmic Kaposi sarcoma cases, 6-16% are eyelid lesions, and the superior and inferior eyelids tend to be involved equally. Of ophthalmic Kaposi sarcoma cases, 7-18% are conjunctival lesions. Many conjunctival lesions tend to involve the inferior conjunctiva (as is shown in the image below) and fornix.
Lesions tend to be indolent, but, as the tumor grows, it can alter ocular adnexal structures [ 14 ] and the ocular surface. The mass effect of the tumor on the eyelids can cause mechanical ectropion or entropion with trichiasis and lagophthalmos and irregular astigmatism. Ectropion or entropion can result in poor lid apposition, trichiasis, and lagophthalmos. Consequently, the patient may experience epiphora, poor tear clearance and drainage, recurrent corneal abrasions, pain and discomfort, foreign body sensation, dry eyes, and photophobia. Long-standing trichiasis and exposure can result in corneal infection, scarring, and opacification.
Rarely, tumor bulk may block the visual axis by ptosis or direct obstruction. Tumor bulk may even prevent the complete closure of the eyelid.
Conjunctival involvement may present with subconjunctival hemorrhage, injection, and chemosis.
Human herpesvirus-8 (HHV-8) DNA or Kaposi sarcoma–associated herpesvirus (KSHV) has been implicated with patients who are HIV-negative or HIV-positive. [ 18 ]
Homosexual males with HIV infection are at an increased risk. This risk is markedly increased with the number of partners.
Patients who have had organ transplants and use immunosuppressive agents and steroids are at an increased risk.
Elderly males of Mediterranean or Ashkenazi ancestry are at an increased risk.
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Meyers D. Eye signs that alert the clinician to a diagnosis of AIDS. SADJ . 2005. 60:386-387. [QxMD MEDLINE Link] .
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[Guideline] Mofenson LM, Brady MT, Danner SP, et al. Guidelines for the Prevention and Treatment of Opportunistic Infections among HIV-exposed and HIV-infected children: recommendations from CDC, the National Institutes of Health, the HIV Medicine Association of the Infectious Diseases Society of America, the Pediatric Infectious Diseases Society, and the American Academy of Pediatrics. MMWR Recomm Rep . 2009 Sep 4. 58:1-166. [QxMD MEDLINE Link] . [Full Text] .
Macken M, Dale H, Moyo D, Chakmata E, Depani S, Israels T, et al. Triple therapy of vincristine, bleomycin and etoposide for children with Kaposi sarcoma: Results of a study in Malawian children. Pediatr Blood Cancer . 2018 Feb. 65 (2): [QxMD MEDLINE Link] .
Hoffmann C, Sabranski M, Esser S. HIV-Associated Kaposi's Sarcoma. Oncol Res Treat . 2017. 40 (3):94-98. [QxMD MEDLINE Link] . [Full Text] .
Brasnu E, Wechsler B, Bron A, et al. Efficacy of interferon-alpha for the treatment of Kaposi's sarcoma herpesvirus-associated uveitis. Am J Ophthalmol . 2005 Oct. 140(4):746-8. [QxMD MEDLINE Link] .
Hummer J, Gass JD, Huang AJ. Conjunctival Kaposi's sarcoma treated with interferon alpha-2a. Am J Ophthalmol . 1993 Oct 15. 116(4):502-3. [QxMD MEDLINE Link] .
Qureshi YA, Karp CL, Dubovy SR. Intralesional interferon alpha-2b therapy for adnexal Kaposi sarcoma. Cornea . 2009 Sep. 28(8):941-3. [QxMD MEDLINE Link] .
Hermans P. Epidemiology, etiology and pathogenesis, clinical presentations and therapeutic approaches in Kaposi's sarcoma: 15-year lessons from AIDS. Biomed Pharmacother . 1998. 52(10):440-6. [QxMD MEDLINE Link] .
Samaniego F, Bryant JL, Liu N, et al. Induction of programmed cell death in Kaposi's sarcoma cells by preparations of human chorionic gonadotropin. J Natl Cancer Inst . 1999 Jan 20. 91(2):135-43. [QxMD MEDLINE Link] .
Simonart T, Noel JC, Andrei G, et al. Iron as a potential co-factor in the pathogenesis of Kaposi's sarcoma?. Int J Cancer . 1998 Dec 9. 78(6):720-6. [QxMD MEDLINE Link] .
Hermans P, Clumeck N, Picard O, et al. AIDS-related Kaposi's sarcoma patients with visceral manifestations. Response to human chorionic gonadotropin preparations. J Hum Virol . 1998 Jan-Feb. 1(2):82-9. [QxMD MEDLINE Link] .
Bailey J, Pluda JM, Foli A, et al. Phase I/II study of intermittent all-trans-retinoic acid, alone and in combination with interferon alfa-2a, in patients with epidemic Kaposi's sarcoma. J Clin Oncol . 1995 Aug. 13(8):1966-74. [QxMD MEDLINE Link] .
Dugel PU, Gill PS, Frangieh GT, Rao NA. Treatment of ocular adnexal Kaposi's sarcoma in acquired immune deficiency syndrome. Ophthalmology . 1992 Jul. 99(7):1127-32. [QxMD MEDLINE Link] .
Korn BS, Park DJ, Kikkawa DO. Intralesional mitomycin-C for the treatment of conjunctival Kaposi sarcoma. Ophthal Plast Reconstr Surg . 2011 Jul-Aug. 27(4):e88-90. [QxMD MEDLINE Link] .
Shuler JD, Holland GN, Miles SA, et al. Kaposi sarcoma of the conjunctiva and eyelids associated with the acquired immunodeficiency syndrome. Arch Ophthalmol . 1989 Jun. 107(6):858-62. [QxMD MEDLINE Link] .
Jackson CC, Dickson MA, Sadjadi M, Gessain A, Abel L, Jouanguy E, et al. Kaposi Sarcoma of Childhood: Inborn or Acquired Immunodeficiency to Oncogenic HHV-8. Pediatr Blood Cancer . 2016 Mar. 63 (3):392-7. [QxMD MEDLINE Link] . [Full Text] .
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 . 1996 Aug. 14(8):2353-64. [QxMD MEDLINE Link] .
Northfelt DW, Dezube BJ, Thommes JA, et al. Efficacy of pegylated-liposomal doxorubicin in the treatment of AIDS-related Kaposi's sarcoma after failure of standard chemotherapy. J Clin Oncol . 1997 Feb. 15(2):653-9. [QxMD MEDLINE Link] .
Welles L, Saville MW, Lietzau J, et al. Phase II trial with dose titration of paclitaxel for the therapy of human immunodeficiency virus-associated Kaposi's sarcoma. J Clin Oncol . 1998 Mar. 16(3):1112-21. [QxMD MEDLINE Link] .
Jacqueline Freudenthal, MD Co-Investigator, Ophthalmic Consultants Centre, Toronto Jacqueline Freudenthal, MD is a member of the following medical societies: American Academy of Ophthalmology , Association for Research in Vision and Ophthalmology , Canadian Ophthalmological Society Disclosure: Nothing to disclose.
Kevin Ryan Yuhan, MD Attending Physician, Cornea, Cataract, Refractive and External Diseases, Southern California Permanente Medical Group Kevin Ryan Yuhan, MD is a member of the following medical societies: Alpha Omega Alpha , American Medical Association , Association for Research in Vision and Ophthalmology , Phi Beta Kappa , American Academy of Ophthalmology , American Society of Cataract and Refractive Surgery Disclosure: Nothing to disclose.
Timothy T You, MD Consulting Surgeon in Ophthalmology, Private Practice Timothy T You, MD is a member of the following medical societies: American Academy of Ophthalmology , American Society of Retina Specialists Disclosure: Nothing to disclose.
Simon K Law, MD, PharmD Clinical Professor of Health Sciences, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology , Association for Research in Vision and Ophthalmology , American Glaucoma Society Disclosure: Nothing to disclose.
Christopher J Rapuano, MD Professor, Department of Ophthalmology, Sidney Kimmel Medical College of Thomas Jefferson University; Director of the Cornea Service, Wills Eye Hospital Christopher J Rapuano, MD is a member of the following medical societies: American Academy of Ophthalmology , American Ophthalmological Society , American Society of Cataract and Refractive Surgery , Contact Lens Association of Ophthalmologists , Cornea Society , Eye Bank Association of America , International Society of Refractive Surgery Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: AAO; OMIC; American Society of Ophthalmic Trauma (ASOT); Avellino, Baxis; Bio-Tissue; Celularity; Dompe; Emmecell; Glaukos; Kala; Oyster Point; Tarsus; TearLab<br/>Serve(d) as a speaker or a member of a speakers bureau for: Dompe<br/>Received research grant from: Glaukos<br/>Received income in an amount equal to or greater than $250 from: AAO; OMIC; Baxis; Bio-Tissue; Cellularity; Dompe; Glaukos; Kala; TearLab<br/>stock options for: RPS, Fount Bio.
Edsel B Ing, MD, PhD, MBA, MEd, MPH, MA, FRCSC Professor, Department of Ophthalmology and Vision Sciences, Sunnybrook Hospital, University of Toronto Faculty of Medicine; Incoming Chair of Ophthalmology, University of Alberta Faculty of Medicine and Dentistry, Canada Edsel B Ing, MD, PhD, MBA, MEd, MPH, MA, FRCSC is a member of the following medical societies: American Academy of Ophthalmology , American Association for Pediatric Ophthalmology and Strabismus , American Society of Ophthalmic Plastic and Reconstructive Surgery , Canadian Medical Association , Canadian Ophthalmological Society , Canadian Society of Oculoplastic Surgery , Chinese Canadian Medical Society, European Society of Ophthalmic Plastic and Reconstructive Surgery, North American Neuro-Ophthalmology Society , Ontario Medical Association , Royal College of Physicians and Surgeons of Canada , Statistical Society of Canada Disclosure: Nothing to disclose.
Anastasios J Kanellopoulos, MD Assistant Program Director, Clinical Associate Professor, Department of Ophthalmology, Manhattan Eye, Ear, and Throat Hospital, New York University
Anastasios J Kanellopoulos, MD is a member of the following medical societies: American Academy of Ophthalmology , Association for Research in Vision and Ophthalmology , Eye Bank Association of America , and International Society of Refractive Surgery
Disclosure: Nothing to disclose.
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The state-of-the-art of mycobacterium chimaera infections and the causal link with health settings: a systematic review.
2. materials and methods, 4. discussion, 4.1. mycobacterium chimaera’s characteristics and ecosystem, 4.2. heater-cooler units, medical devices, water, and air-conditioned implants, 4.3. incubation period and symptoms presentation, 4.4. presence in the lung system, 4.5. modality of transmission, 4.6. detection, 4.7. disinfection, 4.8. causal link assessment, 5. limitations, 6. conclusions, supplementary materials, author contributions, institutional review board statement, informed consent statement, data availability statement, conflicts of interest, abbreviations.
MAC | mycobacterium avium complex |
NTM | non-tuberculosis mycobacterium |
M. chimaera | Mycobacterium chimaera |
HCU | heater-cooler units |
OPPP | opportunistic premise plumbing pathogens |
ECMO | extra-corporal mechanical oxygenation |
HAI | healthcare-associated infection |
Click here to enlarge figure
References | Author, Year | N. of Patients | Surgery | Mean Time of Presentation If Previous Surgery | Setting (Country) | Organ and/or Tissue Involved |
---|---|---|---|---|---|---|
[ ] | (Bills et al., 2009) | 1 | None | Na | Not healthcare (USA) | Lung, nodules in chronic obstructive pulmonary disease |
[ ] | (Cohen-Bacrie et al., 2011) | 1 | None | Na | Possible frequent healthcare contact (Réunion Island, FR) | Lung infections in cystic fibrosis |
[ ] | (Alhanna et al., 2012) | 1 | None | Na | Not healthcare (Germany) | Lung infection |
[ ] | (Gunaydin et al., 2013) | 5 (of 90) | None | Na | Possible healthcare contact (Turkey) | Lung (reassessment of sputum specimens) |
[ ] | (Boyle et al., 2015) | 125 (of 448) | None | Na | Possible healthcare contact (USA) | Lung (reassessment of sputum specimens) |
[ ] | (Mwikuma et al., 2015) | 1 (of 54) | None | Na | Not healthcare (Zambia) | Lung (reassessment of sputum specimens) |
[ ] | (Moon et al., 2016) | 11 | None | Na | Not healthcare (South Korea) | Lung infection (reassessment of sputum specimens) |
[ ] | (Moutsoglou et al., 2017) | 1 | None | Na | Not healthcare (USA) | Disseminated with spinal osteomyelitis and discitis |
[ ] | (Bursle et al., 2017) | 1 | Tricuspid valve repair and mitral annuloplasty | 13 months | Underwent surgery (Australia) | Disseminated |
[ ] | Kim et al., 2017 | 8 (of 91) | None | Na | Possible healthcare contact (Korea) | Lung (reassessment of sputum specimens) |
[ ] | (Chand et al., 2017) * | 4 | Valvular cardiac surgery | 1.15 (0.25–5.1) years | Underwent surgery (UK) | 1 osteomyelitis and 3 disseminated |
[ ] | (Truden et al., 2018) | 49 (of 102) | None | Na | Possible healthcare contact (Slovenia) | Lung (reassessment of sputum specimens) |
[ ] | (Larcher et al., 2019) | 4 | None | Na | Possible frequent healthcare contact (France) | Lung (reassessment of sputum specimens in cystic fibrosis) |
[ ] | (Shafizadeh et al., 2019) * | 5 | Valvular cardiac surgery | 20.6 (14–29) months | Underwent surgery (USA) | Disseminated with liver infection |
[ ] | (Rosero and Shams, 2019) | 1 | None but operating room nurse 10 years ago | >10 years | Possible frequent healthcare contact (USA) | Lung infection |
[ ] | (Watanabe et al., 2020) | 1 | None | Na | Not healthcare (Japan) | Tendons, hand tenosynovitis |
[ ] | (Chen et al., 2020) | 28 | None | Na | Not healthcare (Taiwan) | Lung infection (reassessment of sputum specimens) |
[ ] | (Maalouly et al., 2020) | 1 | Kidney transplantation | One week | Underwent surgery (Belgium) | Kidney, urinary tract infection in a kidney transplant recipient with concomitant Mycobacterium malmoense lung infection and fibro anthracosis |
[ ] | (de Melo Carvalho et al., 2020) | 1 | None | Na | Possible healthcare contact (Portugal) | Disseminated in B-cell lymphoma |
[ ] | (Sharma et al., 2020) | 2 | None | Na | Not healthcare (India) | Meninges, meningitis |
[ ] | (Zabost et al., 2021) | 88 (of 200) | None | Na | Possible healthcare contact (Poland) | Lung infection (reassessment of sputum specimens) |
[ ] | (Kim et al., 2021) | 4 (of 320) | None | Na | Possible healthcare contact (Korea) | Lung infection (reassessment of sputum specimens) |
[ ] | (Kavvalou et al., 2022) | 1 | None | Na | Possible healthcare contact (Germany) | Central venous catheter infection in cystic fibrosis |
[ ] | (Robinson et al., 2022) | 1 | None | Na | Not healthcare (USA) | Lung infection in drug abuser |
[ ] | (Ahmad et al., 2022) | 1 | None | Na | Not healthcare (USA) | Lung infection in sarcoidosis |
[ ] | (George et al., 2022) | 1 | None | Na | Not healthcare (India) | Skin, periapical abscess with chin ulcer |
[ ] | (Lin et al., 2022) | 1 | None | Na | Possible frequent healthcare contact (Taiwan) | Disseminated in adult-onset immunodeficiency syndrome |
[ ] | (Łyżwa et al., 2022) | 1 | None | Na | Not healthcare (Poland) | Lung infection in silicosis |
[ ] | (McLaughlin et al., 2022) | 1 | Coronary artery bypass grafting | 1 year | Underwent surgery (USA) | Tendons, hand tenosynovitis in ipsilateral elbow wound in fisherman |
[ ] | (Gross et al., 2023) | 23 | None | Na | Healthcare (USA) | Lung infections in cystic fibrosis (genomic analysis for cluster correlation to hospital outbreaks) |
[ ] | (Azzarà et al., 2023) | 1 | None | Na | Possible healthcare contact (Italy) | Lung infection in lung adenocarcinoma treated with immune checkpoint inhibitors |
[ ] | (Pradhan et al., 2023) | 1 | Bioprosthetic mitral valve replacement | 7 years | Underwent surgery (Australia) | Spinal osteomyelitis and discitis |
[ ] | (Garcia-Prieto et al., 2024) | 1 | None | Na | Not healthcare (Spain) | Lung infection in fibro anthracosis |
[ ] | (Paul et al., 2024) | 1 | None | Na | Possible healthcare contact (UK) | Lung infection in unilateral pulmonary artery agenesis on the right side |
The statements, opinions and data contained in all publications are solely those of the individual author(s) and contributor(s) and not of MDPI and/or the editor(s). MDPI and/or the editor(s) disclaim responsibility for any injury to people or property resulting from any ideas, methods, instructions or products referred to in the content. |
Bolcato, V.; Bassetti, M.; Basile, G.; Bianco Prevot, L.; Speziale, G.; Tremoli, E.; Maffessanti, F.; Tronconi, L.P. The State-of-the-Art of Mycobacterium chimaera Infections and the Causal Link with Health Settings: A Systematic Review. Healthcare 2024 , 12 , 1788. https://doi.org/10.3390/healthcare12171788
Bolcato V, Bassetti M, Basile G, Bianco Prevot L, Speziale G, Tremoli E, Maffessanti F, Tronconi LP. The State-of-the-Art of Mycobacterium chimaera Infections and the Causal Link with Health Settings: A Systematic Review. Healthcare . 2024; 12(17):1788. https://doi.org/10.3390/healthcare12171788
Bolcato, Vittorio, Matteo Bassetti, Giuseppe Basile, Luca Bianco Prevot, Giuseppe Speziale, Elena Tremoli, Francesco Maffessanti, and Livio Pietro Tronconi. 2024. "The State-of-the-Art of Mycobacterium chimaera Infections and the Causal Link with Health Settings: A Systematic Review" Healthcare 12, no. 17: 1788. https://doi.org/10.3390/healthcare12171788
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Kaposi sarcoma (KS) is an angioproliferative disorder that requires infection with human herpes virus 8 (HHV-8), also known as Kaposi sarcoma-associated herpes virus ... This review will focus on the clinical presentation, staging, diagnosis, and treatment of classic KS. The epidemiology, risk factors, pathology, and molecular pathogenesis of ...
Kaposi sarcoma is an interesting soft tissue tumor occurring in several distinct populations with a variety of presentations and courses. In its most well-known form, Kaposi sarcoma occurs in patients with immunosuppression, such as those with acquired immunodeficiency syndrome (AIDs) or those undergoing immunosuppression due to an organ transplant. Initially described in 1872 by Moritz Kaposi ...
Kaposi sarcoma is a type of cancer that forms in the lining of blood vessels and lymph vessels. The cancer forms growths of cells, called lesions, on the skin. The lesions often form on the face, arms and legs. The lesions may look pink, red, purple or brown. Lesions also can appear on the genitals or in the mouth.
Kaposi Sarcoma. Kaposi sarcoma is a disease in which cancer cells are found in the skin or mucous membranes that line the gastrointestinal (GI) tract, from mouth to anus, including the stomach and intestines. These tumors appear as purple patches or nodules on the skin and/or mucous membranes and can spread to lymph nodes and lungs.
Kaposi sarcoma is a multicentric vascular tumor caused by herpesvirus type 8. It is categorized into 5 types: classic (sporadic), AIDS-associated (epidemic), non-epidemic, endemic (in Africa), and iatrogenic (eg, after organ transplantation). Diagnosis is by biopsy. Treatment for indolent superficial lesions involves cryotherapy ...
This entity occurs in patients with advanced HIV infection and is the most common presentation of Kaposi sarcoma. It is an AIDS-defining cancer, and is approximately 500 times more common in HIV-infected patients than the general US population. Kaposi sarcoma accounts for 12% of cancers in people living with HIV, with 765 to 910 new cases per ...
Kaposi sarcoma (KS) is an angioproliferative disorder that requires infection with human herpesvirus 8 (HHV-8), also known as Kaposi sarcoma-associated herpesvirus ... pathology, and molecular pathogenesis of classic KS. The clinical presentation, diagnosis, staging, and treatment of classic KS is discussed elsewhere, as is AIDS-related KS and ...
1 Summary. Kaposi's sarcoma (KS) most likely originates from lymphatic endothelial cells, and human herpesvirus 8 infection contributes to their malignant transformation. Immunosuppression fosters the occurrence, persistence, and progression of KS, which belongs to the most common AIDS-defining neoplasms in HIV-infected individuals.
Kaposi's sarcoma is the most common tumor associated with human immunodeficiency virus infection and is found in homosexual and bisexual men, injection drug users, and hemophiliacs. ... Clinical Presentation. Natural History of Disease. The natural course of Kaposi's sarcoma can take 1 of 2 forms: (1) a very slowly progressing disease over ...
Kaposi's sarcoma (KS) is a rare angioproliferative tumor classified in four different clinical-epidemiological forms. ... (77.9%) while 5.8% had an exclusively extracutaneous presentation of the disease. Patients presenting with both cutaneous and extracutaneous involvement were 12.2%. Epidemic KS, thus related to HIV-1 and 2, constituted a ...
Kaposi sarcoma (KS) usually appears first as spots (called lesions) on the skin.The lesions can be purple, red, or brown. KS lesions can be flat and not raised above the surrounding skin (called patches), flat but slightly raised (called plaques), or bumps (called nodules).The skin lesions of KS most often develop on the legs or face, but they can also appear in other areas.
Kaposi sarcoma (KS) is a cancer that develops from the cells that line lymph or blood vessels. It usually appears as tumors on the skin or on mucosal surfaces such as inside the mouth, but these tumors can also develop in other parts of the body, such as in the lymph nodes (bean-sized collections of immune cells throughout the body), the lungs ...
Kaposi sarcoma is a disease in which malignant lesions (cancer) can form in the skin, mucous membranes, lymph nodes, and other organs. Kaposi sarcoma is a cancer that causes lesions (abnormal tissue) 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 ...
Kaposi sarcoma typically starts as small, flat patches on the skin or inside the mouth. These patches may be red, purple, pink, brown, black, or blue and look like bruises. They don't usually cause pain at first but can turn into bumps as they grow and may form open wounds. More widespread Kaposi sarcoma can cause: Bleeding or blockage in the ...
Abstract. Kaposi Sarcoma (KS) is the most common neoplasm of people living with HIV today. In Sub-Saharan Africa KS is among the most common cancers in men, overall. Not only HIV+ individuals present with KS; any immune compromised person infected with Kaposi Sarcoma-associated herpesvirus (KSHV) or human herpesvirus 8 is at risk: the elderly ...
Kaposi's sarcoma (KS) is a type of cancer that can form masses on the skin, in lymph nodes, in the mouth, or in other organs. [4] [6] The skin lesions are usually painless, purple and may be flat or raised.[6] [8] Lesions can occur singly, multiply in a limited area, or may be widespread. [6]Depending on the sub-type of disease and level of immune suppression, KS may worsen either gradually or ...
History. AIDS-related Kaposi sarcoma (KS) has a variable clinical course ranging from minimal mucocutaneous disease to widespread organ involvement. The lesions may involve the skin, oral mucosa, lymph nodes, and visceral organs. Most patients present with cutaneous disease. Visceral disease may occasionally precede cutaneous manifestations.
Soft tissue Kaposi sarcoma is a vascular neoplasm caused by human herpesvirus 8 (HHV8); it tends to be indolent but may be locally aggressive. Menu. Chapters By Subspecialty . ... 23 year old HIV+ man with Kaposi sarcoma as a presentation for HIV (Cureus 2021;13:e18936)
Kaposi's sarcoma is characterized by the appearance of purplish, reddish-blue, or dark brown/black macules, plaques, or nodules on the skin. ... (African), immunosuppression related and acquired immunodeficiency syndrome (AIDS) associated. In the cutaneous presentation is most often on the distal lower extremities the classic Kaposi's and ...
Kaposi's sarcoma (KS) is a multi-focal, angioproliferative neoplasm that usually appears on the skin, but can also involve the visceral organs. In 1981, 26 cases of KS were reported as occurring in young homosexual men in New York and California. Many of these patients also had concomitant pneumocystis carinii pneumonia (PCP) and a variety of ...
Research and Statistics. See the estimates for cases of Kaposi sarcoma in the US and what research is currently being done. Key Statistics About Kaposi Sarcoma. What's New in Kaposi Sarcoma Research? About Kaposi Sarcoma. Causes, Risk Factors, and Prevention. Early Detection, Diagnosis, and Staging. Treatment.
Of ophthalmic Kaposi sarcoma cases, 6-16% are eyelid lesions, and the superior and inferior eyelids tend to be involved equally. Of ophthalmic Kaposi sarcoma cases, 7-18% are conjunctival lesions. Many conjunctival lesions tend to involve the inferior conjunctiva (as is shown in the image below) and fornix. The inferior conjunctiva is involved ...
(1) Background. A definition of healthcare-associated infections is essential also for the attribution of the restorative burden to healthcare facilities in case of harm and for clinical risk management strategies. Regarding M. chimaera infections, there remains several issues on the ecosystem and pathogenesis. We aim to review the scientific evidence on M. chimaera beyond cardiac surgery, and ...