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Aortic angiosarcoma clinically mimicking polyarteritis nodosa.

A Sparsa1, E Liozon, J Wechsler

  • 1Departments of Dermatology, University Hospital of Limoges, France.

Scandinavian Journal of Rheumatology
|June 13, 2006
PubMed
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Angiosarcoma of the aorta (ASA) can mimic polyarteritis nodosa (PAN), leading to misdiagnosis. Early identification of ASA is crucial for accurate treatment and improved patient outcomes.

Area of Science:

  • Vascular Oncology
  • Pathology
  • Cardiovascular Surgery

Background:

  • Angiosarcoma of the aorta (ASA) is a rare and aggressive malignancy.
  • Distal emboli and skin metastases are potential complications of ASA.
  • Systemic necrotizing vasculitis, such as polyarteritis nodosa (PAN), shares overlapping clinical features with ASA.

Observation:

  • Two patients presented with symptoms mimicking PAN, including constitutional symptoms, leg pain, skin necrosis, and elevated inflammatory markers.
  • Initial biopsies were inconclusive or suggested vasculitis, with transient improvement following corticosteroid treatment.
  • Subsequent investigations, including factor VIII-positive atypical endovascular cell proliferation on skin biopsy and intra-luminal aortic tumor on MRA, confirmed ASA.

Findings:

Related Experiment Videos

  • ASA can present with a clinical picture highly suggestive of PAN, leading to diagnostic delays.
  • The "PAN-like" manifestations in these cases were predominantly localized to the lower body.
  • Factor VIII reactivity in atypical endovascular cells is a key diagnostic clue for ASA.

    • Implications:

    • Clinicians should consider ASA in the differential diagnosis of patients presenting with vasculitis-like symptoms, especially with distal emboli or skin lesions.
    • Advanced imaging like MRA is essential for diagnosing intra-aortic tumors.
    • Timely diagnosis of ASA is critical for appropriate management and potentially improving prognosis.