Child with rapidly progressive unilateral proptosis, displacement of the globe inferiorly, and edema of upper eyelid?
Rhabdomyosarcoma
- most common primary orbital malignancy of childhood
- malignant growth of striated muscle tissue
- rapidly progressive mass in the superior orbit with proptosis, globe displacement, and eyelid swelling
- average age of presentation is 7 years
- Prompt diagnosis with orbitotomy and biopsy is crucial
- overall mortality is 60% once the disease has extended to orbital bones
- Current Rx with radiation + chemo have lowered mortality rates to 5 to 10%