Kaposi's Sarcoma: a Clinico-Pathological Study of 8 Patients

Jang, Kyoung Ae; Chi, Doo Hyun; Choi, Jee Ho; Sung, Kyung Jeh; Moon, Kee Chan; Koh, Jai Kyoung

Abstract

BACKGROUND: Kaposi's sarcoma (KS) is a multicentric neoplastic vascular tumor involving the skin or internal organs. KS is an extremely common tumor in tropical Africa and it is also a prime marker of acquired immunodeficiency syndrome (AIDS). Nearly all cases of KS can be classified in four groups: 1) classic, 2) African, endemic, 3) AIDS-associated, 4) iatrogenic immunosuppression- associated KS.
OBJECTIVE
The purposes of this study were aimed at evaluating the clinical and biological features of KS.
METHODS
The hospital charts and histopathologic slides of patients with KS diagnosed at Asan Medical Center from 1989 to 1999 were reviewed. Immunohistochemical studies for factor VIII, CD31, and CD34 were performed. We also performed PCR-based analysis to determine whether human herpesvirus 8 (HHV8) is present in KS included in this study.
RESULTS
1. Four male and four female patients were included. The mean age at diagnosis of KS was 59.6 years. 2. Our patients could be classified as follows, iatrogenic immunosuppression-associated (7/8) and classic KS (1/8). Iatrogenic immunosuppression-associated group was sub-classified into long-standing steroid use (2/7), chemotheraphy-associated (cancer related, 3/7), and kidney transplantation (KT)-associated group (2/7). Patients with AIDS-associated KS were absent. 3. KS was presented with varied clinical features showing from ill-defined purplish macules to fungating nodules or tumors. Interestingly, two cases were presented with zosteriform or cellulitis-like features. Although upper and especially, lower extremities were favored sites, mucosal involvement such as intraoral and perianal areas was noticed. The cases showing the involvement of systemic organ could not be detected except two cases involving the small intestine. 4. Immunohistochemical studies revealed that spindle cells in stroma were partially positive for CD31 and CD34 and negative for factor VIII. 5. HHV8 sequences were identified in all cases of KS. 6. Four patients (50%) died within 1-2 years after the diagnosis of KS due to underlying diseases. Radiation therapy and interferon-alpha were effective in one case, respectively. Classic KS showed indolent course.
Conclusion
KS is a multicentric neoplasm for which the etiopathogenesis is still under discu ssion. We retrospectively studied seven cases with iatrogenic immunosuppression-associated KS and one with classic KS. KS should be considered when the cutaneous lesions newly developed in immunosuppressed patients. HHV8 may contribute to the pathogenesis of KS when other predisposing conditions are present.