Temporal bone rhabdomyosarcoma mimicking otitis media complicated by facial nerve palsy

Ahmed Galal,Omneya Ahmed,Rania G Aly,Hanan Yehia Tayel,Azza M Rizk

doi:10.1186/s43163-021-00126-5

Abstract

BackgroundRhabdomyosarcoma is common in childhood, especially, the head and neck region, yet involvement of the temporal bone is rare.Case presentationWe reported a case of an embryonal rhabdomyosarcoma in a 4.5-year-old boy presenting with external auditory canal polyp and purulent otorrhea that later developed grade 6 facial palsy. Imaging showed soft tissue mass involving the middle ear, mastoid cavity, parotid gland, and parapharyngeal space. Subtotal petrosectomy with blind closure of the external auditory canal was performed with facial nerve decompression and debulking biopsy followed by combined chemoradiation.ConclusionMiddle ear rhabdomyosarcoma is a rare pathology, usually present in childhood by symptoms similar to suppurative otitis media not responding to medical treatment leading to delayed diagnosis and development of complications.

Highlights

Rhabdomyosarcoma is common in childhood, especially, the head and neck region, yet involvement of the temporal bone is rare.Case presentation: We reported a case of an embryonal rhabdomyosarcoma in a 4.5-year-old boy presenting with external auditory canal polyp and purulent otorrhea that later developed grade 6 facial palsy
About 30–50% of cases present in the head and neck area [5, 6], with only 3% presenting within the middle ear (ME) or the temporal bone (TB) which is occasionally associated with cranial nerves affection [4, 6]
Before 1972, the mainline of treatment was surgical extirpation followed by radiotherapy till the Intergroup Rhabdomyosarcoma Study (IRS) protocol was brought out, which advised the multi-agent chemoradiation as the first line of treatment and surgery only is preserved to provide tissue biopsy, stop discharge, or when Facial nerve (FN) decompression is required [14]

Summary

Conclusion

Rhabdomyosarcoma is a common pediatric neoplasm, rare in the middle ear and mastoid cavity. Its presentation is deceiving in this region, since it would mimic complicated otitis media presenting with polyps, discharge which is not responding to medical treatment, and occasionally facial palsy. This might lead to delayed diagnosis until metastasis has occurred which might worsen the prognosis. Neurological signs, and a firm postauricular swelling might be suggestive of a neoplasm requiring CT. The role of surgery in these neoplasms is only biopsy, debridement, stoppage of discharge, and decompression of the facial nerve when required and not radical excision with curative intent

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