What is classic (sporadic) Kaposi sarcoma (KS)?

Updated: Apr 11, 2019
  • Author: Jessica Katz, MD, PhD, FACP; Chief Editor: Edwin Choy, MD, PhD  more...
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Answer

This entity typically occurs primarily in elderly men of Mediterranean and Eastern European background. It has a male predominance with a male-to-female ratio of 10-15:1. The age of onset is between 50 and 70 years.

Classic Kaposi sarcoma usually follows a protracted and indolent course. Common complications include venous stasis and lymphedema. This form of the disease rarely includes lymph node, mucous membrane, or visceral involvement. [31]

As many as 30% of patients with classic Kaposi sarcoma subsequently develop a second malignancy, typically a non-Hodgkin lymphoma. [31] These second malignancies may be due to immune suppression from age, host genetics, history of other neoplasm, and possible concurrent infections such as malaria. [32, 33, 34]

Contradictory evidence suggests that immune activation is requisite for the development of classic Kaposi sarcoma. Indeed both hypotheses may be correct, [17, 18] indicating a complicated mechanism of immune dysregulation.

Infrequent bathing; a history of asthma; and, in men, a history of allergy have been linked to classic Kaposi sarcoma in Italian patients. [35]  Long-term use of topical steroids is associated with increased risk, either indicating an effect of chronic dermatitis or of the steroids themselves. As in epidemic AIDS-related Kaposi sarcoma, a protective effect of cigarette smoking has also been noted.


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