A mistaken identity: rhabdomyosarcoma of the middle ear cleft misdiagnosed as chronic suppurative otitis media with temporal lobe abscess

Abstract

SUMMARYA 5-year-old girl presented with a 3-month history of left side facial palsy, followed sequentially by purulent ear discharge, complete external ophthalmoplaegia and blurred vision. On clinical examination she was febrile with left-sided conductive hearing loss. She was clinically diagnosed to have chronic suppurative otitis media of the unsafe type with petrous apicitis, middle cranial fossa abscess and cavernous sinus involvement. Preliminary CT scan findings were reported as a large left temporal lobe abscess and left otitis media with cholesteatoma. MRI of the brain obtained later corroborated the abnormalities detected on the CT scan. Ten days after admission, a mass was seen protruding from the external auditory canal. A biopsy of the mass was obtained and sent for histopathological examination. Meanwhile, review of the MRI suggested an aggressive neoplasm such as sarcoma/ rhabdomyosarcoma. Histopathology clinched the final diagnosis of an anaplastic type of embryonal rhabdomyosarcoma of the middle ear cleft. BACKGROUND

Diagnosis and Treatment of Nodular Fasciitis of Ear Region in Children: A Case Report and Review of Literature

“…The biggest study was the retrospective review of 50 cases of auricular NF done by Thompson et al [ 4 ]; at presentation the mean age was 27 years but there was no info about the age at the onset of the disease. The remaining eight articles were two retrospective studies and six case reports, which described a total of thirteen pediatric patients (7 males and 4 females); two cases did not report sex [ 13 , 16 ]) from 17 months until 18 years old of age [ 6 , 7 , 8 , 13 , 14 , 15 , 16 ].…”

“…To maximize the results and improve the long-term free-of-disease period, different surgical approaches have been proposed based on the location and position of the lesion [ 7 , 8 , 13 , 14 , 15 ].…”

“…Rarely NF localized in the ear region was reported in literature [ 4 , 5 ] and more rarely described in pediatric age [ 6 , 7 ]. Masses in the external ear can be due to rhabdomyosarcoma [ 8 ], angioleiomyoma [ 9 ], fibroepithelial polyp [ 10 ], squamous cell carcinoma [ 11 ], otitis malignant and suppurate tissue in case of persistence of an external body in the EAC [ 12 ]. The diagnostic management includes ultrasonography (US), computed tomography (CT) and magnetic resonance imaging (MRI) [ 7 ].…”

Rhabdomyosarcoma of the External Ear Canal Treated with Brachytherapy

“…[6] This delay can result in tumor growth causing cranial nerve deficits, as well as meningeal or brain parenchyma involvement. [7] It is not unusual for RMS of the ear to present concurrently with facial nerve palsies and loss of taste at the anterior 2/3 of the tongue. Our patient fortunately was diagnosed early in the course of the disease (within 10 days of initial symptomatology), which may have prevented further involvement of surrounding structures.…”