CA: A Cancer Journal for Clinicians, Vol 31, 2-12, Copyright
© 1981 by American Cancer Society
Kaposi's Sarcoma: A Review and Recent Developments
Bijan Safai M.D.1 and
Robert A. Good Ph.D., M.D.2
1 Associate Attending Physician, Chief, Dermatology Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center, New York, New York.
2 Vice President, Memorial Sloan-Kettering Cancer Center, New York, New York.
Since it was first described in 1872, KS has presented challenging questions to physicians and investigators. The possible influence of infectious, genetic, or environmental factors in KS is suggested by the cluster distribution of the disease; this has been recognized only during the past two decades. The infrequency of familial KS and the lack of consanguinity noted argue against a genetic origin as the sole factor involved. Investigation of the role of major histocompatibility antigens in KS is needed and might reveal a genetic influence.
There are already strong indications for the seroepidemiologic association of CMV with KS. Further work is necessary to fully determine the roles of CMV and environmental factors in the pathogenesis of this disease. The rarity of familial cases suggests that even if the disease does involve an infectious agent, it is not contagious as such, but is a result of multiple factors at work. An appealing view is that a slow-growing virus infecting individuals with a particular genetic or immunologic makeup, or both, may lead to their developing KS.
Although the basic process underlying the close association of KS with other cancers is still unknown, this finding suggests related etiopathogenic mechanisms. The association of KS especially with lymphoreticular malignancies suggests that common factors may be operating in a susceptible host, and that there may be shared tumor inducers or promoters in each of these several diseases.
The nature and origin of the cell involved have not been established. Cultivation of KS tumors in immunodeficient laboratory animals seems to offer a logical approach to providing evidence of the neoplastic nature of the disease and for selecting out which is the actual malignant cell.
Decreased immune functions and impaired immunosurveillance in individuals with a certain genetic makeup and exposure to certain environmental agents may trigger the process whereby KS cells develop and expand. Thus, further investigations into the possible participation of altered immune functions in the development of KS may help toward an understanding of this unusual disease.
