Kaposi Sarcoma

Description

Kaposi Sarcoma: An idiopathic pigmented sarcoma of the skin. Red nodules of varying size develop in the skin initially appearing on the sole of the foot then affecting the hands, progressing to affect the rest of the body.

Kaposi’s original description is now one branch of a more widely recognised disease that has developed over the last century.

History

1869  – Köbner identified a skin sarcoma that occurred as primary skin disorder in a case report, having previously only described those thought to be secondary to an internal malignancy. [1869;1(3):369-381.]

1872  – Kaposi presented five cases, treated between 1868 and 1871, of primary skin sarcomas that followed similar clinical (and histological) patterns. With no underlying cause or malignancy he termed these ‘Idiopathic pigmented sarcoma of the skin’. In the cases presented, all men over 40 years old, the course of the disease was fast and terminal. [1872;4:265-73]

Nodules the size of peas or hazelnuts, brown-red to blue-red in colour. They are either isolated or form groups; in the latter case, the central nodules of a plaque retrogress and generate a pitted, dark-pigmented depression. They usually appear first on the feet, then on the hands. As the disease progresses, isolated nodules and groups of nodules appear on the arms, legs, face and trunk. Finally, identical nodules appear on the mucous linings of the larynx, trachea, stomach, intestines and liver. The disease is rapidly lethal, with death within two or three years.‘ – Kaposi 1872

Kaposi argued that although it was possible that the disease process was initially local and spread lymphatically, he thought this unlikely ‘as the disease starts almost simultaneously on both hands and feet, and as for this reason we must assume the existence of a general disease from the start, we can not hope to prevent the lethal progress by early extirpation of the first nodules‘

1950’s – First descriptions of African KS; a fulminant and more severe form of the disease which rapidly progressed to the patient’s death.

1970’s – Immunotherapy associated KS, seen in Renal transplant patients and others diseases requiring immunosuppressive agents. In most cases, the disease as seen to regress on ceasing the offending drugs.

1981 – The fourth, and most widely recognised form, is in association with AIDS. Widespread sarcomas, coexisting with opportunistic infections and precipitously fatal.

Associated Persons

• Moriz Kaposi (1837-1902)
• Heinrich Köbner (1838-1904)

Alternative names

• Idiopathic pigmented sarcoma of the skin
• KS

References

• Köbner H. Zur Kenntniss der allgemeinen Sarcomatose und der Hautsarcome im Besonderen. [To understand sarcomatosis in general and skin sarcoma in particular] Archiv für Dermatologie und Syphilis. 1869;1(3):369-381.
• Kaposi M. Idiopathisches multiples pigmentsarkom der Haut. Archiv für Dermatologie und Syphilis. 1872;4:265-73. [Re-print/translation: Idiopathic multiple pigmented sarcoma of the skin. CA Cancer J Clin. 1982 Nov-Dec;32(6):340-7. PMID 6812893]
• Shiels RA. A history of Kaposi’s sarcoma. J R Soc Med. 1986 Sep; 79(9): 532–534. [PMC1290459]
• Harwood AR, Osoba D, Hofstader SL, Goldstein MB, Cardella CJ, Holecek MJ, Kunynetz R, Giammarco RA. Kaposi’s sarcoma in recipients of renal transplants. Am J Med. 1979 Nov;67(5):759-65. [PMID 159624]
• Friedman-Kien AE, Laubenstein LJ, Rubinstein P, Buimovici-Klein E, Marmor M, Stahl R, Spigland I, Kim KS, Zolla-Pazner S. Disseminated Kaposi’s Sarcoma in Homosexual Men. Ann Intern Med. 1982 Jun;96(6 Pt 1):693-700. [PMID 6283973]