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Case Report

Aortic angiosarcoma clinically mimicking polyarteritis nodosa

, , , , , , , & show all
Pages 237-240 | Received 25 May 2005, Accepted 11 Nov 2005, Published online: 12 Jul 2009
 

Abstract

We report two patients in whom angiosarcoma of the aorta (ASA) with distal emboli and skin metastases was initially clinically misdiagnosed as polyarteritis nodosa (PAN). Both presented with features highly suggestive of systemic necrotizing vasculitis, including constitutional symptoms, orchitis (in one), leg pain with sensory neuropathy, livedo, focal areas of skin necrosis, painful cutaneous nodules demonstrating vasculitis or inconspicuous changes on biopsy, raised acute phase reactants, and transient improvement with systemic corticosteroids. Repeated skin biopsies revealed an atypical endovascular cell proliferation with strong reactivity for factor VIII. Magnetic resonance angiography (MRA) demonstrated an intra‐luminal aortic tumour at the infra‐renal level. In retrospect, the (PAN)‐like features appeared to involve exclusively the lower part of the body in these patients.

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