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Case Study: May 2012
History
- 9 year old male.
- 2 year history of mass in orbit.
- Non-axial proptosis.
- Orbital biopsy.
- Patient otherwise fit and well.
- See histology figures.
Questions
- What do the H&E’s show?
- Which immunostains have been used in figs 4 an 5?
- What is the diagnosis?
- What further tests can be done to confirm the diagnosis?
- What is the significance of the finding in Fig 5?
Click to show answers
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- Spindle cell lesion with chronic inflammatory cells.
- Fig 4 is an ALK-1 and Fig 5 is an IgG4
- Inflammatory myofibroblastic tumour.
- Chromosomal translocation testing for presene or absence of activation of the ALK tyrosine kinase. 50% of paediatric cases are positive.
- This is an incidental finding and does not indicate IgG4 disease.
References
- 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. - 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
