[A rapidly progressive orbital tumor in a seven-year-old girl revealing a primitive systemic vasculitis]

Ann Pathol. 2014 Dec;34(6):469-73. doi: 10.1016/j.annpat.2014.09.007. Epub 2014 Nov 15.
[Article in French]

Abstract

Orbital tumor lesions in pediatric population encompass a wide range of pathological processes, which are very different in etiology and prognosis. They usually require an emergency in histological diagnosis because of their quick growth. Beside malignant and benign tumors, the inflammatory pseudo-tumors group included dysimmune orbital involvement secondary to systemic vasculitis such as granulomatosis with polyangiitis (ex-Wegener's granulomatosis). We report the case of a seven-year-old girl suffering from a severe primitive ANCA vasculitis, revealed by an orbital mass.

Keywords: ANCA vasculitis; Children; Enfant; Granulomatose avec polyangéite (maladie de Wegener); Granulomatosis with polyangiitis (Wegener's granulomatosis); Orbital pseudo-tumor; Pseudo-tumeur orbitaire; Vascularite à ANCA.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic / blood
  • Asthma / complications
  • Bronchitis / complications
  • Child
  • Disease Progression
  • Female
  • Glomerulonephritis, Membranoproliferative / etiology
  • Granulomatosis with Polyangiitis / complications
  • Granulomatosis with Polyangiitis / diagnosis*
  • Granulomatosis with Polyangiitis / immunology
  • Humans
  • Laryngostenosis / etiology
  • Orbital Pseudotumor / etiology*
  • Orbital Pseudotumor / pathology

Substances

  • Antibodies, Antineutrophil Cytoplasmic