Rare Infectious Disease News

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Kaposi sarcoma

Kaposi sarcoma is an interesting soft tissue tumor occurring in several distinct populations. In its most well-known form, Kaposi sarcoma occurs in patients with immunosuppression, such as those with acquired immunodeficiency syndrome

Prevelance

6 / 1 000 000

1,980

US Estimated

3,078

Europe Estimated

Age of Onset

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ICD-10

C46.0
C46.1
C46.2
C46.3
C46.7
C46.8
C46.9

Inheritance

Autosomal dominant

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Autosomal recessive

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Mitochondrial/Multigenic

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X-linked dominant

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X-linked recessive

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Rare View

5 Facts you should know

FACT

1

First described by Moritz Kaposi in 1872

FACT

2

KS causes red or purple lesions to grow under the skin and in the lining of the mouth, nose, and throat

FACT

3

KS is caused by a combination of immune suppression and infection by Human herpesvirus 8 (KSHV)

FACT

4

Over 35% of people with AIDS may be affected by Kaposi’s sarcoma

FACT

5

 More common in older men of Jewish or Mediterranean descent

Kaposi sarcoma is also know as...

Kaposi sarcoma

KS

What’s your Rare IQ?

This famous fashion designer passed away from HIV-related Kaposi's sarcoma in 1990.

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

(Brand name: Votrient) Manufactured by GlaxoSmithKline
FDA-approved indication: Advanced soft tissue sarcoma (STS) who have received prior chemotherapy.

Top Clinical Trials

TitleDescriptionPhasesStatusInterventionsMore Information
Pomalidomide Treatment in Patients With Kaposi SarcomaThis 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 2RecruitingDrug: PomalidomideClick here for more information
Trial of Ixazomib for Kaposi SarcomaThis 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 2RecruitingDrug: Ixazomib Citrate|Other: Quality-of-Life Assessment|Other: Questionnaire AdministrationClick here for more information
A Study of sEphB4-HSA in Kaposi SarcomasEphB-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 2RecruitingDrug: sEphB4-HSAClick here for more information
Abemaciclib in Patients With HIV-associated and HIV-negative Kaposi SarcomaKaposi 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 2RecruitingDrug: AbemaciclibClick here for more information
NHS-IL12 Monotherapy and in Combination With M7824 in Advanced Kaposi SarcomaKaposi 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 2RecruitingDrug: NHS-IL12|Drug: NHS-IL12+M7824Click here for more information
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 2RecruitingDrug: Zidovudine|Drug: Liposomal Doxorubicin|Drug: Valganiclovir|Drug: Rituximab|Other: Standard TherapiesClick here for more information
sEphB4-HSA in Treating Patients With Kaposi SarcomaThis 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 2RecruitingOther: Laboratory Biomarker Analysis|Other: Pharmacological Study|Behavioral: Quality-of-Life Assessment|Biological: Recombinant EphB4-HSA Fusion ProteinClick here for more information
Nelfinavir Mesylate in Treating Patients With Kaposi SarcomaThis 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 2RecruitingOther: Laboratory Biomarker Analysis|Drug: Nelfinavir MesylateClick here for more information
Virotherapy and Natural History Study of KHSV-Associated Multricentric Castleman s Disease With Correlates of Disease ActivityThis 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 2RecruitingDrug: 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 OnlyClick here for more information
Testing CC-486 (Oral Azacitidine) Plus the Standard Drug Therapy in Patients 75 Years or Older With Newly Diagnosed Diffuse Large B Cell LymphomaThis 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 3RecruitingDrug: Cyclophosphamide|Drug: Doxorubicin Hydrochloride|Drug: Oral Azacitidine|Drug: Prednisone|Other: Questionnaire Administration|Biological: Rituximab|Drug: Vincristine SulfateClick here for more information

Top Treatments in Research

AgentClass/Mechanism of ActionDevelopment StatusCompanyClinical StudiesMore 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 2National Cancer Institute (NCI)Click here for more informationClick here for more information
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 2AIDS Malignancy Consortium|National Cancer Institute (NCI)Click here for more informationClick here for more information
sEphB4-HSAA 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 2Vasgene Therapeutics, IncClick here for more informationClick here for more information
AbemaciclibAbemaciclib 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 2National Cancer Institute (NCI)|National Institutes of Health Clinical Center (CC)Click here for more informationClick here for more information
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 2National Cancer Institute (NCI)|National Institutes of Health Clinical Center (CC)Click here for more informationClick here for more information
Nelfinavir MesylateNelfinavir 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 2AIDS 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 CenterClick here for more informationClick here for more information