Rhabdomyosarcoma of the middle ear cavity presenting with Horner’s syndrome

Abstract

Rhabdomyosarcoma is a rare malignant tumour of striated muscle accounting for 3% of all primary soft tissue tumours of the middle ear cavity. We describe a unique case of a rhabdomyosarcoma of the middle ear cavity in a four-year-old boy, presenting as an otherwise asymptomatic ipsilateral Horner’s syndrome. This case involved tumour erosion through the tensor tympani semicanal, accessing the carotid canal and the sympathetic plexus running through it. This child was treated successfully with chemotherapy and proton beam therapy, and remains disease free at six years of follow-up. This case highlights the importance of investigating patients with non-resolving ear infections, polyps or granulation tissue.