Treatment of the classic form is palliative and includes local radiation therapy and chemotherapy. The type of cell that gives rise to Kaposi's sarcoma has not been clearly established. The frequent occurrence of primary lymphomas in patients with this illness suggests a reticuloendothelial origin. Histologic features include hyperemic vascular slits between spindle-shaped cells, often in association with extravasated erythrocytes, hemosiderin, nuclear pleomorphism, and fibrosis. Kaposi's sarcoma shows an ethnic predominance, appearing most often in Eastern Europeans, Italians, Russians, and Jews.
Familial cases are rare.
The disease may be endemic in equatorial Africa. An aggressive lymphadenopathic form of Kaposi's sarcoma appears frequently in this region, especially among children. Evidence suggests a role for viruses in different forms of Kaposi's sarcoma. The geographic distribution of Kaposi's sarcoma in Africa corresponds to that of Burkitt's lymphoma, which is closely associated with the Epstein-Barr virus. The occurrence of Kaposi's sarcoma in AIDS patients and in renal transplant patients receiving immunosuppressive therapy further suggests that altered immune function plays a role in the development of this disease.