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Rheumatology Advance Access originally published online on April 2, 2008
Rheumatology 2008 47(5):645; doi:10.1093/rheumatology/ken115
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© The Author 2008. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Aortic angiosarcoma mimicking large-vessel vasculitis: a diagnostic dilemma

M. Abdin-Mohamed, J. Ledingham, F. Witham and F. McCrae

Department of Rheumatology, Queen Alexandra Hospital, cosham, Portsmouth, UK

Correspondence to: M. Abdin-Mohamed. E-mail: maysamabdin{at}hotmail.com

A 55-yr-old lady presented with myalgia, arthralgia and claudicant thigh pain. Examination showed mildly reduced global power and reduced distal leg pulses. Investigations revealed grossly elevated inflammatory markers. Further detailed investigations were normal except abdominal CT and angiogram that showed almost complete occlusion of the distal aorta supporting a working diagnosis of a large-vessel vasculitis (LVV).

Sequential treatment with prednisolone, infusions of cyclophosphamide and methylprednisolone and insertion of an aortic stent resulted in only minor symptomatic improvement and continued elevation in blood inflammatory markers. Despite infliximab for a working diagnosis of treatment-resistant LVV, new symptoms of left tibial pain and a raised serpiginous rash occurred. Left tibial imaging revealed a destructive osteolytic lesion. Skin and bone biopsies revealed a high-grade multi-focal epithelioid angiosarcoma.

This lady died despite chemotherapy and palliative radiotherapy. A post-mortem confirmed the diagnosis of primary aortic angiosarcoma (PAA).

PAA is extremely rare (30 cases reported in the literature). It has a very poor prognosis and most commonly originates in the descending thoracic or abdominal aorta [1]. This case highlights the diverse presentations of malignancies and the high index of suspicion required with cases of vasculitis that are poorly responsive to conventional treatments.

Disclosure statement: The authors have declared no conflicts of interest.

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  1. Schipper J, Van Oostayen JA, Den Hollander JC, Van Seyen AJ. Aortic tumours: report of a case and review of the literature. Br J Radiol (1989) 62:35–40.[Abstract/Free Full Text]
Accepted 15 February 2008


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This Article
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