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Case Reports

Abstract

An 87-year-old Ukrainian male presented with three violaceous papules on the left third toe. A shave biopsy demonstrated a nodular proliferation of spindle cells forming slit-like vascular spaces with prominent erythrocyte extravasation. Immunohistochemical staining for HHV-8 was positive, confirming nodular-stage Kaposi sarcoma (KS). Two weeks later, the patient presented with a bluish subcutaneous nodule on the right lower leg. Punch biopsy sections showed a mid-dermal vascular lesion with robust erythrocyte extravasation and spindle cells. The spindle cells were irregularly shaped and pleomorphic with occasional prominent nucleoli and scattered mitoses. CD31 and CD34 highlighted the vascular lesion. HHV-8 stains showed granular positivity, consistent with KS. The patient was HIV-negative and not otherwise immunocompromised. KS in HIV-negative patients and in those of Caucasian or Ukrainian descent is highly unusual. In fact, only 27% of cases occur outside of Africa, and 21% occur in patients without HIV. Our patient’s presentation is consistent with classic KS, a variant that affects Eastern European or Mediterranean older men. Of note, the patient spent time in a concentration camp during childhood and was exposed to Agent Orange in the military, which may have caused long-term immune dysregulation and modestly increased overall cancer risk. We seek to highlight the importance of biopsy of vascular lesions in elderly patients with military exposure but no history of immunosuppression, as classic KS carries a favorable prognosis with appropriate treatment compared to other types. Our patient had a CT scan without evidence of metastasis and is planning to undergo local radiotherapy.