A 4-year-old boy presents with rapid onset unilateral nasal obstruction and epistaxis. Examination shows a greyish, grape-like mass filling the left nasal cavity with proptosis. Biopsy shows small round cells with cross-striations and positive staining for desmin and myogenin. The most appropriate first-line treatment is:
- A Multimodal chemotherapy with radiotherapy, surgery reserved for residual disease ✓
- B Wide local excision alone
- C Radical excision followed by brachytherapy
- D Observation after biopsy confirmation
Correct answer: A. Multimodal chemotherapy with radiotherapy, surgery reserved for residual disease
Explanation
This is embryonal rhabdomyosarcoma, the most common malignant tumour of the nasal cavity and paranasal sinuses in young children. Desmin and myogenin positivity confirms myogenic differentiation. Because these tumours are highly chemosensitive, standard management is multiagent chemotherapy with radiotherapy, with surgery reserved for residual or recurrent disease, which allows organ preservation. Surgery-first approaches yield poor local control and observation leads to rapid dissemination.
Reference: Nelson Textbook of Pediatrics, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.