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Case Reports
. 2019 Jan;101(1):e8-e10.
doi: 10.1308/rcsbull.2019.8. Epub 2018 Oct 5.

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

S M Hayes  1 , T C Biggs  2 , J H Bird  3 , W P Hellier  2
Affiliations
Case Reports

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

S M Hayes et al. Ann R Coll Surg Engl. 2019 Jan.

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.

Keywords: Chronic suppurative otitis media; Horners syndrome; Middle ear; Rhabdomyosarcoma.

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Figures

Figure 1
Figure 1
Axial computed tomography showing complete opacification of the right middle ear cleft with bony destruction of the right carotid canal (asterisk).
Figure 2
Figure 2
Medium-powered photomicrograph showing sub-epithelial atypical cells including occasional large hyperchromatic nuclei adjacent to an area of necrosis. (haematoxylin and eosin × 20).
Figure 3
Figure 3
Immunohistochemically stained photomicrograph with desmin (left) and myogenin (right) highlighting atypical cells beneath the epithelium, diagnosing rhabdomyosarcoma (× 20).
Figure 4
Figure 4
Axial magnetic resonance image through the mastoid, revealing no evidence of disease recurrence at five years.
See this image and copyright information in PMC

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