Case Study: May 2012


  • 9 year old male.
  • 2 year history of mass in orbit.
  • Non-axial proptosis.
  • Orbital biopsy.
  • Patient otherwise fit and well.
  • See histology figures.


  1. What do the H&E’s show?
  2. Which immunostains have been used in figs 4 an 5?
  3. What is the diagnosis?
  4. What further tests can be done to confirm the diagnosis?
  5. What is the significance of the finding in Fig 5?
Click to show answers
  1. Spindle cell lesion with chronic inflammatory cells.
  2. Fig 4 is an ALK-1 and Fig 5 is an IgG4
  3. Inflammatory myofibroblastic tumour.
  4. Chromosomal translocation testing for presene or absence of activation of the ALK tyrosine kinase. 50% of paediatric cases are positive.
  5. This is an incidental finding and does not indicate IgG4 disease.


  1. Inflammatory myofibroblastic tumour of the orbit in a 7-year-old child.
    Ahmad SM, Tsirbas A, Kazim M.
    Clin Experiment Ophthalmol. 2007 Mar;35(2):160-2.
  2. gG4 plasma cells in inflammatory myofibroblastic tumor: inflammatory marker or pathogenic link?
    Saab ST, Hornick JL, Fletcher CD, Olson SJ, Coffin CM.
    Mod Pathol. 2011 Apr;24(4):606-12