Case Study: December 2011


  • 5 year old male child
  • Left non-axial proptosis developed over 2 months
  • Biopsy performed of orbital mass
  • See figures 1 and 2


  1. What do Figures 1 and 2 show? What is the differential diagnosis?
  2. The tumour was strongly positive for CD56, chromogranin, synaptophysin, PGP9.5 and for NSE. What are the likeliest diagnoses now?
  3. What could you suggest to the ophthalmologists?
Click to show answers
  1. Packeted small round blue cell tumour. Differential is : rhabdomyosarcoma, extraocular retinoblastoma, neuroblastoma (olfactory v metastatic), Wilm’s, Ewing’s, PNET.
  2. Olfactory v metastatic neuroblastoma. Olfactory would be unusual in a child though.
  3. Abdominal imaging. In this case, the child had a left suprarenal mass. The diagnosis was therefore one of orbital metastatic neuroblastoma.