Facial paralysis: a presenting feature of rhabdomyosarcoma

International Journal of Pediatric Otorhinolaryngology 46 (1998) 221 – 224

Case report

Facial paralysis: a presenting feature of rhabdomyosarcoma Mohammed M.S. Jan * College of Medicine and Allied Health Sciences, Jeddah, Saudi Arabia Received 29 April 1998; received in revised form 8 August 1998; accepted 11 August 1998

Abstract The purpose of this paper is to present a child with embryonal rhabdomyosarcoma involving the left middle ear, who initially presented with unilateral facial paralysis. A 5-year-old boy presented with a 4-week history of left-sided facial weakness, associated with persistent otitis media on that side. Examination revealed complete left lower motor neuron facial weakness and hearing loss. A myringotomy revealed a soft tissue mass behind the tympanic membrane. Biopsy and oncologic assessment confirmed a stage II, group III left middle ear embryonal rhabdomyosarcoma. Despite debulking surgery, local irradiation and multiple chemotherapeutic courses the child deteriorated quickly. He developed carcinomatous meningitis and died 9 months after his initial presentation. In conclusion, middle ear tumors should be considered in the differential diagnosis of unresolving otitis media, particularly when associated with persistent ipsilateral facial paralysis. An ear mass, discharge, facial swelling, or systemic symptoms may be initially absent despite the presence of this aggressive malignancy. Careful examination of the middle ear is recommended in children with facial weakness. A myringotomy incision may be necessary including a complete assessment of the middle ear cavity, particularly when there is no fluid return. © 1998 Published by Elsevier Science Ireland Ltd. All rights reserved. Keywords: Facial paralysis; Rhabdomyosarcoma; Malignancy

1. Introduction

* Present address: The Department of Pediatrics (Neurology), King Abdulaziz University Hospital, P.O. Box 6615, Jeddah 21452, Saudi Arabia. Tel.: +966 2 6401000; fax: +966 2 695 2538.

Facial nerve paralysis, or Bell’s palsy, is a benign neuropathy of the seventh cranial nerve, usually of unknown cause [1]. The incidence in children below the age of 10 years is 2.7 per 100000 [2]. Facial nerve paralysis is an uncommon but well described complication of chronic

0165-5876/98/$ - see front matter © 1998 Published by Elsevier Science Ireland Ltd. All rights reserved. PII S0165-5876(98)00112-8

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suppurative otitis media [3]. The surgical management of this complication is controversial, however, surgery for decompressing the nerve and hasten recovery has been suggested [4]. Middle ear tumors are rare in children [5,6]. The usual presenting features include; chronic otitis media, ear discharge, cervical lymphadenopathy, ear mass, hearing loss and facial asymmetry [6 – 9]. Rhabdomyosarcoma is the most common soft tissue malignancy seen in childhood [10]. It originates from skeletal muscles and has three major subtypes; embryonal, alveolar and pleomorphic. Approximately 50% of all rhabdomyosarcomas in children occur in the head and neck region, however, the ear is rarely involved [11]. In a series of 24 patients with malignant tumors of the ear, only three had rhabdomyosarcoma [6]. Rhabdomyosarcoma of the ear is highly aggressive and has poor prognosis in most cases [6,8,9,12]. In this paper, the case of a child with unilateral facial paralysis and otitis media is presented. A myringotomy revealed a middle ear embryonal rhabdomyosarcoma.

A myringotomy was performed without return of pus following an antero–superior incision. Extension of the incision revealed a soft tissue mass behind the tympanic membrane. Biopsy made a diagnosis of embryonal rhabdomyosarcoma. A CT scan showed a left middle ear mass with some bony destruction, but without intracranial extension (Figs. 1 and 2). An oncologic assessment confirmed a stage II, group III left middle ear embryonal rhabdomyosarcoma. The patient underwent tympano–mastoidectomy for tumor debulking. During the surgery the facial nerve was not visualized because of the vascular nature of the tumor. Despite local irradiation and multiple chemotherapeutic courses the child deteriorated quickly. He developed carcinomatous meningitis and died 9 months after his initial presentation.

3. Discussion This patient illustrates the aggressive nature of middle ear embryonal rhabdomyosarcoma. The initial diagnosis of idiopathic Bell’s palsy was changed to facial palsy secondary to otitis media

2. Case history A 5-year-old boy presented with a 4-week history of left-sided facial weakness. One week following the onset of his facial palsy, a diagnosis of left otitis media was made and he was treated with oral antibiotics. Because of the persistence of the facial weakness he was assessed by a pediatric neurologist 4 weeks after the onset. Examination revealed a well looking child. He had no fever but his left tympanic membrane was bulging and opaque. The right ear was normal and there was no mastoid tenderness on either side. On neurologic examination, he had complete left lower motor neuron facial weakness and hearing loss on the left. The remainder of his assessment was normal. Urgent consultation with the pediatric otorhinolaryngologist was made, who recommended immediate myringotomy. Tympanometry, audiometry and facial nerve conduction studies were not performed.

Fig. 1. CT scan of the middle ear showing opacification of the left middle ear with some bony destruction.

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weakness. A myringotomy incision may be necessary, including a complete assessment of the middle ear cavity, particularly when there is no fluid return.

Acknowledgements I wish to thank Dr Margaret Yhap and Dr Joseph M. Dooley for their helpful advice during writing this article and for critically reviewing the final manuscript.

References

Fig. 2. CT scan at the level of the mastoid bone showing opacification and some bony destruction.

1 week later, when a diagnosis of an ipsilateral ear infection was made. The persistence of otitis media, despite antibiotic therapy and facial paralysis, after 4 weeks resulted in referral to a pediatric neurologist and otolaryngologist. The diagnosis of embryonal rhabdomyosarcoma was made at the time of myringotomy. Two similar cases, with middle ear rhabdomyosarcoma and Bell’s palsy, were reported in the non-English language [13,14]. The first case had a polyp in the external auditory canal which led to early diagnosis [13]. The second case had persistent otorrhea [14]. The patient presented herein did not have any local or systemic symptoms to indicate an underlying malignancy. Specifically, he did not have an ear polyp, mass, discharge, or facial swelling. The only suggestion of his diagnosis was his facial paralysis. To conclude, middle ear tumors should be considered in the differential diagnosis of unresolving otitis media, particularly when associated with persistent ipsilateral facial paralysis. An ear mass, discharge, facial swelling, or systemic symptoms may be initially absent despite the presence of this aggressive malignancy. Careful examination of the middle ear is recommended in children with facial

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