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Kaposi sarcoma
Kaposi's sarcoma is a type of cancer caused by the human herpes virus 8. It appears as red or purple patches on the skin, mouth, lungs, liver, or digestive system
Prevalence
6 / 1 000 000
Age of Onset
ICD-10
C46.0
C46.1
C46.2
C46.3
C46.7
C46.8
C46.9
Inheritance
This condition does not appear to have a clear pattern of inheritance.
Rare View
Kaposi sarcoma (KS) is a cancer that causes patches of abnormal tissue to grow under the skin, in the lining of the mouth, nose, and throat, in lymph nodes, or in other organs. The lesions are usually red or purple, are composed of cancer cells, blood vessels, and blood cells. KS is caused by infection with human herpesvirus-8 (HHV-8).
5 Facts you should know
FACT
First described by Moritz Kaposi in 1872
FACT
KS causes red or purple lesions to grow under the skin and in the lining of the mouth, nose, and throat
FACT
KS is caused by a combination of immune suppression and infection by Human herpesvirus 8 (KSHV)
FACT
Over 35% of people with AIDS may be affected by Kaposi’s sarcoma
FACT
More common in older men of Jewish or Mediterranean descent
Interest over time
Google searches
Common signs & symptoms
Recurrent herpes
Susceptibility to herpesvirus
Abnormality of the lower limb
Lower limb deformities
Hypermelanotic macule
Hyperpigmented spots
Neoplasm of the skin
Skin tumors
Abnormal retinal morphology
Retina issue
Abnormality of the gastrointestinal tract
Abnormality of the spleen
Encephalopathy
Current treatments
The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.
Daunorubicin citrate liposome injection
(Brand name: DaunoXome) Manufactured by Gilead
FDA-approved indication: First line cytotoxic therapy for advanced, HIV related Kaposi's sarcoma.
Interferon alfa-2b (recombinant)
(Brand name: Intron A) Manufactured by Schering-Plough
FDA-approved indication: Treatment of selected patients with AIDS-related Kaposi's sarcoma.
Alitretinoin
(Brand name: Panretin (topical)) Manufactured by Eisai Medical Research, Inc
FDA-approved indication: Topical treatment of cutaneous lesions in patients with AIDS-related Kaposi's sarcoma.
Interferon alfa-2a (recombinant)
(Brand name: Roferon®-A) Manufactured by Hoffmann-La Roche, Inc.
FDA-approved indication: Treatment of chronic myelogenous leukemia.
Paclitaxel
(Brand name: Taxol) Manufactured by Bristol-Myers Squibb Co
FDA-approved indication: For the second line treatment of AIDS-related Kaposi's sarcoma.
Pazopanib
FDA-approved indication: Advanced soft tissue sarcoma (STS) who have received prior chemotherapy.
Top Clinical Trials
Title | Description | Phases | Status | Interventions | More Information |
---|---|---|---|---|---|
Pomalidomide Treatment in Patients With Kaposi Sarcoma | This phase II trial studies the effect of pomalidomide in treating patients with Kaposi sarcoma. Pomalidomide is a cancer fighting drug that stops the growth of blood vessels, stimulates the immune system, and may kill cancer cells. | Phase 2 | Recruiting | Drug: Pomalidomide | More Info |
Trial of Ixazomib for Kaposi Sarcoma | This phase II trial studies how well ixazomib works in treating patients with Kaposi sarcoma. Ixazomib may stop the growth of tumor cells by blocking some of the enzymes needed for cell growth. | Phase 2 | Recruiting | Drug: Ixazomib Citrate|Other: Quality-of-Life Assessment|Other: Questionnaire Administration | More Info |
A Study of sEphB4-HSA in Kaposi Sarcoma | sEphB-HSA may prevent tumor cells from multiplying and blocks several compounds that promote the growth of blood vessels that bring nutrients to the tumor. The purpose of this study is to learn if sEphB4-HSA will decrease the number or size of Kaposi sarcoma lesions in people. | Phase 2 | Recruiting | Drug: sEphB4-HSA | More Info |
Abemaciclib in Patients With HIV-associated and HIV-negative Kaposi Sarcoma | Kaposi Sarcoma (KS) is common in people with human immunodeficiency virus (HIV) but can also occur in people who do not have HIV. KS tumors usually involve the skin, but may also involve lymph nodes, lungs, bone, and gastrointestinal tract. Researchers want to see if a drug that is currently used to treat a type of breast cancer can help. To find a safe dose of abemaciclib to treat KS and to see if it can shrink lesions or tumors. | Phase 1|Phase 2 | Recruiting | Drug: Abemaciclib | More Info |
NHS-IL12 Monotherapy and in Combination With M7824 in Advanced Kaposi Sarcoma | Kaposi sarcoma (KS) tumors grow on the skin, lymph nodes, lungs, bone, and gastrointestinal tract. KS often affects people with immune deficiencies, such as among people living with HIV or those with prior history of transplant. Researchers want to see if 2 non-chemotherapy drugs can help people with KS. NHS-IL12 triggers the immune system to fight tumors. M7824 blocks the pathways that cancer cells use to stop the immune system from fighting tumors. The objective of this study is to learn if giving NHS-IL12 alone or with M7824 could help the immune system fight KS tumors. | Phase 1|Phase 2 | Recruiting | Drug: NHS-IL12|Drug: NHS-IL12+M7824 | More Info |
History of the KSHV Inflammatory Cytokine Syndrome (KICS) | KSHV inflammatory cytokine syndrome (KICS) is a newly recognized disease caused by Kaposi sarcoma-associated herpesvirus (KSHV). This virus can cause cancer. People with KICS can have severe symptoms. They include fever, weight loss, and fluid in the legs or abdomen. People with KICS may also be at risk of getting other cancers associated with KSHV. These cancers include Kaposi sarcoma and lymphoma. Because KICS is a newly identified disease, more information is needed on how the disease works and what can be done to treat it. The objctive of this study is to collect genetic and medical information from people with KSHV inflammatory cytokine syndrome. | Phase 2 | Recruiting | Drug: Zidovudine|Drug: Liposomal Doxorubicin|Drug: Valganiclovir|Drug: Rituximab|Other: Standard Therapies | More Info |
sEphB4-HSA in Treating Patients With Kaposi Sarcoma | This phase II trial studies recombinant EphB4-HSA fusion protein (EphB4-HSA) in treating patients with Kaposi sarcoma. Recombinant EphB4-HSA fusion protein may block the growth of blood vessels that provide blood to the cancer, and may also prevent cancer cells from growing. | Phase 2 | Recruiting | Other: Laboratory Biomarker Analysis|Other: Pharmacological Study|Behavioral: Quality-of-Life Assessment|Biological: Recombinant EphB4-HSA Fusion Protein | More Info |
Nelfinavir Mesylate in Treating Patients With Kaposi Sarcoma | This pilot phase II trial studies how well nelfinavir mesylate works in treating patients with kaposi sarcoma. Nelfinavir mesylate may stop the growth of tumor cells by blocking some of the enzymes needed for cell growth. | Phase 2 | Recruiting | Other: Laboratory Biomarker Analysis|Drug: Nelfinavir Mesylate | More Info |
Virotherapy and Natural History Study of KHSV-Associated Multricentric Castleman s Disease With Correlates of Disease Activity | This study will gain information about a rare disorder called KSHV-associated multicentric Castleman s disease (MCD). KSHV, a virus, causes several kinds of cancer, including some forms of MCD. KSHV stands for the Kaposi s sarcoma herpes virus, also called human herpes virus-8, or HHV-8. Researchers want to understand the biology of KSHV-MCD to identify how this disease causes illness and to find ways to treat it. There is no standard therapy effective for all cases of KSHV-MCD. The disease is often fatal, and about half the people who have it die within 2 years of diagnosis. | Phase 2 | Recruiting | Drug: Etoposide|Drug: Interferon-alpha|Drug: Rituximab|Drug: Zidovudine|Drug: Liposomal Doxorubicin|Drug: Bortezomib|Drug: Valganciclovir|Drug: Doxorubicin|Drug: Vincristine|Drug: Cyclophosphamide|Drug: Filgrastim (G-CSF)|Drug: Prednisone|Drug: Sirolimus|Other: Observation Only | More Info |
Testing CC-486 (Oral Azacitidine) Plus the Standard Drug Therapy in Patients 75 Years or Older With Newly Diagnosed Diffuse Large B Cell Lymphoma | This phase II/III trial compares the side effects and activity of oral azacitidine in combination with the standard drug therapy (reduced dose rituximab-cyclophosphamide, doxorubicin, vincristine, and prednisone [R-miniCHOP]) versus R-miniCHOP alone in treating patients 75 years or older with newly diagnosed diffuse large B cell lymphoma. R-miniCHOP includes a monoclonal antibody (a type of protein), called rituximab, which attaches to the lymphoma cells and may help the immune system kill these cells. R-miniCHOP also includes prednisone which is an anti-inflammatory medication and a combination of 3 chemotherapy drugs, cyclophosphamide, doxorubicin, and vincristine. These 3 chemotherapy drugs, as well as oral azacitidine, work in different ways to stop the growth of cancer cells, either by killing the cells, by stopping them from dividing, or by stopping them from spreading. Combining oral azacitidine with R-miniCHOP may shrink the cancer or extend the time without disease symptoms coming back or extend patient's survival when compared to R-miniCHOP alone. | Phase 2|Phase 3 | Recruiting | Drug: Cyclophosphamide|Drug: Doxorubicin Hydrochloride|Drug: Oral Azacitidine|Drug: Prednisone|Other: Questionnaire Administration|Biological: Rituximab|Drug: Vincristine Sulfate | More Info |
Top Treatments in Research
Agent | Class/Mechanism of Action | Development Status | Company | Clinical Studies | More Information |
---|---|---|---|---|---|
Pomalidomide | In vitro, POMALYST has been demonstrated to inhibit the proliferation and induce apoptosis of hematopoietic tumor cells and to inhibit the proliferation of lenalidomide-resistant multiple myeloma cell lines. Pomalidomide is also believed to have immunomodulatory activity - enhanced T cell- and natural killer cell-mediated immunity. | Phase 2 | National Cancer Institute (NCI) | More Info | More Info |
Ixazomib Citrate | At therapeutic concentrations, ixazomib selectively and reversibly inhibits the protein proteasome subunit beta type-5 (PSMB5) with a dissociation half-life of 18 minutes. | Phase 2 | AIDS Malignancy Consortium|National Cancer Institute (NCI) | More Info | More Info |
sEphB4-HSA | A soluble decoy EphB4 receptor-human serum albumin fusion protein (sEphB4-HSA) binds to EphrinB2 and blocks interaction with the cell surface EphB4 receptor to promote immune infiltration and induce tumor cell death. | Phase 2 | Vasgene Therapeutics, Inc | More Info | More Info |
Abemaciclib | Abemaciclib is an antitumor agent and dual inhibitor of cyclin-dependent kinases 4 (CDK4) and 6 (CDK6) that are involved in the cell cycle and promotion of cancer cell growth in case of unregulated activity. | Phase 1|Phase 2 | National Cancer Institute (NCI)|National Institutes of Health Clinical Center (CC) | More Info | More Info |
NHS-IL12 | IL-12 plays an important role in the activities of natural killer cells and T lymphocytes. IL-12 mediates enhancement of the cytotoxic activity of NK cells and CD8+ cytotoxic T lymphocytes. There also seems to be a link between IL-2 and the signal transduction of IL-12 in NK cells. IL-2 stimulates the expression of two IL-12 receptors, IL-12R-β1 and IL-12R-β2, maintaining the expression of a critical protein involved in IL-12 signaling in NK cells. Enhanced functional response is demonstrated by IFN-γ production and killing of target cells. IL-12 also has anti-angiogenic activity, which means it can block the formation of new blood vessels. It does this by increasing production of interferon-gamma, which in turn increases the production of a chemokine called inducible protein-10 (IP-10 or CXCL10). IP-10 then mediates this anti-angiogenic effect. Because of its ability to induce immune responses and its anti-angiogenic activity, there has been an interest in testing IL-12 as a possible anti-cancer drug. However, it has not been shown to have substantial activity in the tumors tested to this date. | Phase 1|Phase 2 | National Cancer Institute (NCI)|National Institutes of Health Clinical Center (CC) | More Info | More Info |
Nelfinavir Mesylate | Nelfinavir binds to the protease active site and inhibits the activity of the enzyme. This inhibition prevents cleavage of the viral polyproteins resulting in the formation of immature non-infectious viral particles. Protease inhibitors are almost always used in combination with at least two other anti-HIV drugs. | Phase 2 | AIDS Malignancy Consortium|National Cancer Institute (NCI)|The Emmes Company, LLC|University of Arkansas|University of California, Los Angeles|AIDS and Cancer Specimen Resource|Montefiore Medical Center | More Info | More Info |