Angiosarcoma complicating lower leg elephantiasis in a male patient: An unusual clinical complication, case report and literature review

Abstract

Chronic lymphedema is rarely complicated by an angiosarcoma. Angiosarcoma superimposed on chronic lymphedema (Stewart-Treves syndrome) is usually seen post breast cancer surgery accompanied by lymph node resection of the axilla. This is a case report of a 59-year-old male patient with elephantiasis that developed an angiosarcoma of the lower leg. He died a month after the diagnostic biopsy was obtained. This is a rare multifocal tumor in a male with an unusual lower leg location. We reviewed the literature and the need to differentiate this often deadly lesion from a Kaposi's sarcoma.

Keywords: Elephantiasis; Kaposi’s sarcoma; Stewart–Treves syndrome; angiosarcoma; lower extremity lymphedema.

Figure 1.

Right leg lymphedema, sero-hemorrhagic drainage, notice extensive fibrinous and eschar tissue (before debridement and antibiotic therapy).

Figure 2.

Right leg 1-week after debridement and under broad-spectrum intravenous antibiotic therapy.

Figure 3.

Highly atypical and pleomorphic neoplastic cells form solid aggregations and rudimentary vascular structures (H&E, upper) and strongly express CD31 (immunostaining, lower).

Figure 4.

These atypical cells strongly express CD31 immunostaining (lower), and stained positively for vimentin, CD34 typical of epithelioid sarcoma (angiosarcoma) but negative for Kaposi sarcoma–associated human herpesvirus-8 (HHV-8), pankeratin (AE1/AE3), p63, CK7, CK20, S100, SOX10 and CD45.