Non-AIDS associated Kaposi's sarcoma: clinical features and treatment outcome

Abstract

Background: Kaposi's sarcoma (KS) in HIV negative patients is rare and has to be distinguished from AIDS associated KS. Two groups are at risk to develop non-AIDS related KS: elderly men mainly of Mediterranean origin and persons with iatrogenic immunosuppression.

Patients and methods: In order to define risk-groups and major clinical features we retrospectively evaluated clinical data of all patients with non-AIDS associated KS presenting to the Department of Dermatology, University Hospital Tuebingen between 1987 and 2009. Data were extracted from the tumor registry of the Comprehensive Cancer Center Tuebingen and from patient records.

Results: 20 patients with non-AIDS KS have been identified. The average age at KS onset was 66.6 years; the male-to-female-ratio was 3:1. Most of the patients were immigrants from Mediterranean or Eastern European countries (60%). 15 cases of classic KS versus 5 cases of iatrogenic KS were observed. In 95% of the cases, KS was limited to the skin, without mucosal, lymph node or visceral manifestation. KS lesions were in all cases multiple and mostly bilateral, the most common localization was the skin of the lower extremities. Tumor control was achieved in nearly all cases by the use of local or systemic therapy. No patient died from KS.

Conclusions: Unlike KS in AIDS patients, non-AIDS associated KS is a rather localized process which rarely involves lymph nodes or organs. It is mostly seen in elderly males from Mediterranean or Eastern European countries and in most cases responsive on local or systemic therapeutic strategies.

Figure 1. Brownish macules and plaques on the foot of a patient with classic KS.

Figure 2. Patch stage classic KS: Red to brownish irregularly-shaped macules and plaques.

Figure 3. Patch stage KS with promontory sign.

Dilated irregular vascular channels surround a pre-existing vessel.

Figure 4. Plaque stage KS with bizarre vessels dissecting the upper dermis.

There is erythrocyte extravasation and hemosiderin pigmentation.

Figure 5. Tumor stage KS: Well-circumscript spindle-cell tumor.

Erythrocytes lie within poorly defined slit-like vascular spaces.

Figure 6. Tumor stage KS: Close up view.

Figure 7. D2-40 staining of arborizing vascular structures; irregular lymphatic vessels.

Figure 8. HHV-8 staining of atypical endothelial cells.