Kaposi sarcoma

Other Names: Kaposi's sarcoma; Mediterranean Kaposi sarcoma; Non AIDS related Kaposi sarcoma A type of cancer in which lesions (abnormal areas) grow in the skin, lymph nodes, lining of the mouth, nose, and throat, and other tissues of the body.

Kaposi's sarcoma

Summary

The lesions are usually purple and are made of cancer cells, new blood vessels, and blood cells. They may begin in more than one place in the body at the same time. Kaposi sarcoma is caused by Kaposi sarcoma-associated herpes virus (KSHV). In the United States, it usually occurs in people who have a weak immune system caused by AIDS or by drugs used in organ transplants. It is also seen in older men of Jewish or Mediterranean descent, or in young men in Africa.

Kaposis sarcoma

Pathophysiology

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 tumors can also develop in other parts of the body (including the lymph nodes, lungs, or digestive tract). The abnormal cells of Kaposi sarcoma cause purplish, reddish blue, or dark brown/black skin lesions (macules, nodules, plaques) on the legs and the face. These lesions may look bad, but they usually cause no symptoms. However, when the lesions are in the lungs, liver, or digestive tract, they may cause serious problems like gastrointestinal bleeding or trouble breathing.[1] Kaposi sarcoma is caused by infection with a virus called the Kaposi sarcoma associated herpesvirus (KSHV), also known as human herpesvirus 8 (HHV8).[2] Kaposi sarcoma is classified into four types based upon the different populations in which it develops: classic (which presents in middle or old age), endemic (described in sub-Saharan indigenous Africans), iatrogenic (associated with immunosuppressive drug therapy) and AIDS-associated (epidemic KS).[3] Options for treatment may include local therapy, radiation therapy, chemotherapy and biologic therapy (immunotherapy). The main aim is to restore immunity.

Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Patch stage Kaposi's sarcoma

Diagnosis

Making a diagnosis for a genetic or rare disease can often be challenging. Healthcare professionals typically look at a person’s medical history, symptoms, physical exam, and laboratory test results in order to make a diagnosis. The following resources provide information relating to diagnosis and testing for this condition. If you have questions about getting a diagnosis, you should contact a healthcare professional.

Testing Resources

The Genetic Testing Registry (GTR) provides information about the genetic tests for this condition. The intended audience for the GTR is health care providers and researchers. Patients and consumers with specific questions about a genetic test should contact a health care provider or a genetics professional.

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Kaposi sarcoma

Treatment

FDA-Approved Treatments The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.

• 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.(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.

Conditions with similar signs and symptoms from Orphanet

The differential diagnosis may include bacillary angiomatosis, hemosiderotic hemangioma, fibrous histiocytoma, interstitial granuloma annulare, arteriovenous malformations, and pyogenic granuloma.

External links

• Kaposi sarcoma photo library at Dermnet

Classification	ICD-10: C46 ICD-9-CM: 176 OMIM: 148000 MeSH: D012514 DiseasesDB: 7105
External resources	MedlinePlus: 000661 eMedicine: med/1218 derm/203 oph/481