Temporal bone rhabdomyosarcoma in children

Differentiating Pediatric Rhabdomyosarcoma and Langerhans Cell Histiocytosis of the Temporal Bone by Imaging Appearance

“…10 Chronic otitis media, polyps of the external auricular canal, and retroauricular masses are all common pre- sentations. 3,10 Other series showed a much higher prevalence of neurologic signs, including a series in which 9 of 14 patients presented with facial nerve palsies. 10 Among patients with RMS lesions of the temporal bone, 50% had lesions that were localized to a specific temporal bone subsite, and the remaining 50% had extratemporal extension (n ϭ 6).…”

“…These diseases are distinct pathologically: RMS is a soft-tissue sarcoma arising from mesenchymal cells of the middle ear or skeletal muscle, 1 while LCH tumors arise from the abnormal proliferation of Langerhans cells (ie, dendritic cells). 2 Both lesions are commonly found in the head and neck region, 30%-70% in RMS 3 and 60% in LCH. 4 Less frequently, the temporal bone is involved, with only 8%-10% of cases of RMS of the head and neck 3 and 19%-25% of LCH cases in this location.…”

“…2 Both lesions are commonly found in the head and neck region, 30%-70% in RMS 3 and 60% in LCH. 4 Less frequently, the temporal bone is involved, with only 8%-10% of cases of RMS of the head and neck 3 and 19%-25% of LCH cases in this location. 4 The initial presentation for both diseases is variable and can range from a palpable periauricular mass or external auricular canal polyps to nonspecific symptoms such as hearing loss, otalgia, or otorrhea.…”

“…4 The initial presentation for both diseases is variable and can range from a palpable periauricular mass or external auricular canal polyps to nonspecific symptoms such as hearing loss, otalgia, or otorrhea. 3,4 Some patients may be completely asymptomatic. 5 Because these diseases can easily mimic benign processes, the diagnoses of RMS and LCH are usually only considered after imaging reveals a skull base or temporal bone lesion.…”

“…10 Among patients with RMS lesions of the temporal bone, 50% had lesions that were localized to a specific temporal bone subsite, and the remaining 50% had extratemporal extension (n ϭ 6). 3 The staging system for RMS classifies a T1 lesion as being limited to 1 subsite of the temporal bone (mastoid, middle ear, or petrous apex). 11 The middle ear is considered an unfavorable subsite for RMS staging.…”