Background: Classic Kaposi's sarcoma (CKS) is not uncommon in Greece with a reported incidence of 0.20 per 100,000 per year.
Methods: Epidemiological, clinical and histological features of all CKS cases, diagnosed in 'A. Sygros' hospital, Athens, Greece during the years 1989-1994, have been recorded and studied prospectively.
Results: During the five-year period studied, 66 CKS patients have been diagnosed in our hospital. Incidence among dermatologic patients was 2.11 per 10,000 patients examined, representing 1.35% of total skin malignancies. Patients' age at diagnosis ranged from 53 to 94 years (mean 72 +/- 8.8). The male to female ratio was 2.47:1. A high proportion of the patients were born in Peloponnesos (42.42%) and were residing in Athens (51.51%) or in Peloponnesos (24.24%). Nodules and/or plaques were the most frequent type of lesion, most commonly located on the feet (43.93%) or the hands (28.78%). Accompanying edema was seen in 51.51% of the patients. There were 16 stage I patients (24.24%), 40 stage II (60.60%), 0 stage III and 10 stage IV (15.15%). Involvement of visceral organs was detected in seven patients (10.60%), while 10 had lymph node involvement (15.15%) and three, involvement of the underlying bones (4.54%). Second primary malignancy was diagnosed in 6 cases (9.09%), most often of the reticuloendothelial system (83.33%).
Conclusions: CKS in Greece exhibits some special characteristics, including older age of onset; lower male to female ratio; endemic clustering; disseminated skin disease at diagnosis, often accompanied by lymphedema; not unusual visceral or lymph node involvement and association with second malignancies. We suggest that CKS in Greece possibly represents a distinct endemic subtype of CKS.