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Non-AIDS Kaposi sarcoma
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Non-AIDS Kaposi sarcoma

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Contributors: Ivy Lee MD, Belinda Tan MD, PhD, Lynn McKinley-Grant MD, Aída Lugo-Somolinos MD, Lowell A. Goldsmith MD, MPH
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Synopsis

Kaposi sarcoma (KS) is a malignant neoplasm of lymphatic endothelial cell origin that occurs in several forms: AIDS-associated (discussed separately) and non-AIDS-related, including classic KS, endemic KS, and iatrogenically-induced KS. All 4 types can be linked to coinfection with human herpesvirus type 8 (HHV-8), and the cutaneous lesions are morphologically and histologically indistinguishable among the types. KS may be a reactive process rather than a true neoplasm because it does not produce conventional metastases but spreads in a multifocal way. 

Classic (traditional) KS is seen almost exclusively in people of Mediterranean and Ashkenazi Jewish descent, with age of onset typically between 50 and 70 years. Older literature described classic KS occurring more often in males, with a male-to-female ratio of approximately 12:1. However, more recent studies suggest that the sex gap may not be so significant.

Classic Kaposi sarcoma most commonly runs an indolent course for 10-15 years or more, with slow enlargement of tumors and the gradual development of additional lesions. After many years, systemic lesions can develop along the gastrointestinal tract, in lymph nodes, and in other organs. These visceral tumors are usually asymptomatic. Up to one-third of the patients with classic KS develop a second primary malignancy, most frequently non-Hodgkin lymphoma.

There is an aggressive endemic form in equatorial black African individuals that is also unrelated to AIDS. Areas of highest prevalence include Uganda, the Congo Republic, Zambia, and Congo (Brazzaville). Systemic lesions are particularly prevalent in this form. The male-to-female ratio is nearly equal among children but increases with age.

Iatrogenic KS is the result of long-term systemic immunosuppression and is common in organ transplant recipients, especially renal. It may resolve when immunosuppressive medications are discontinued. This variant is mostly limited to the skin, with infrequent visceral involvement.

Codes

ICD10CM:
C46.0 – Kaposi's sarcoma of skin

SNOMEDCT:
109386008 – Kaposi's sarcoma of skin

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Updated: 10/29/2018
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Non-AIDS Kaposi sarcoma
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Non-AIDS Kaposi sarcoma : Acral, Purple color, Feet, Lower legs, Smooth nodules
Clinical image of Non-AIDS Kaposi sarcoma
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