Suspect orbital tumours where the vignette involves a child or middle-aged woman, with proptosis.
Childhood Orbital Tumours
Tumour |
Description |
Key features |
|---|---|---|
Rhabdomyosarcoma |
Commonest primary childhood orbital malignancy. Most commonly affects the genitourinary system and the head and neck |
Typically presents on the superonasal orbit. Suspect in children with rapidly progressive painless unilateral proptosis, with down and out displacement of the eyeball |
Neuroblastoma |
Tumor of neural crest cells that can metastasise to the orbit from elsewhere in the body. |
Histology shows characteristic Homer-wright rosettes |
Capillary Hemangioma |
High-flow vascular hamartomas which grow rapidly in infancy and completely resolve at around the age of 8. |
Presents as a blanching red unilateral lesion of the upper lid If small and no risk of affecting the visual axis, observation is sufficient. If there is a risk of amblyopia, propranolol can be used to increase the rate of lesion regression. |
Optic nerve glioma |
Tumour of the glial tissue of the optic nerve, associated with NF1 |
Presents as slowly progressing unilateral proptosis with visual defects and RAPD in children CT shows characteristic fusiform enlargement of the optic nerve |
Adulthood Orbital Tumours
Tumour |
Description |
Presentation |
Investigations |
|---|---|---|---|
Optic nerve sheath meningioma |
Tumour of the meningeal tissue of the optic nerve |
Presents with a triad of painless unilateral vision loss, optic nerve atrophy and optociliary shunting of vessels, in a middle-aged woman |
CT shows a thick optic nerve sheath and osteoblastic change |
Cavernous Hemangioma |
Low-flow hamartoma within the common tendinous ring |
Presents with slowly progressive axial proptosis and decreased visual acuity in a middle-aged woman |
CT shows defined lesion within the common tendinous ring |
