A 4-year-old boy develops rapidly progressive unilateral proptosis over 3 weeks with ptosis and downward displacement of the globe. CT shows a poorly defined superonasal orbital soft tissue mass eroding adjacent bone. Biopsy confirms small round blue cells positive for desmin and MyoD1. First-line management is:
- A Immediate radical orbital exenteration
- B Observation with serial imaging
- C Chemotherapy followed by radiotherapy, with surgery reserved for residual disease ✓
- D Local steroid injection
Correct answer: C. Chemotherapy followed by radiotherapy, with surgery reserved for residual disease
Explanation
Embryonal rhabdomyosarcoma is the commonest primary malignant orbital tumour of childhood, presenting as rapidly progressive proptosis. It is chemosensitive and radiosensitive, so current protocols use multiagent chemotherapy (vincristine, actinomycin D, cyclophosphamide) plus radiotherapy, reserving exenteration only for residual or recurrent disease. Observation is fatal, and steroids are used for orbital inflammation, not this tumour.
Reference: Kanski's Clinical Ophthalmology, 9th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.