Paediatric rhabdomyosarcoma of the ear and temporal bone

Abstract

The objective of the present study was to review the presentation, management, outcome and morbidity of paediatric patients presenting to a single centre with rhabdomyosarcoma of the ear and temporal region. All patients diagnosed with rhabdomyosarcoma of the ear and temporal region between 1980 and 2000 were entered into this retrospective study. Fourteen patients were identified. The median age at presentation was 4.5 years with a mean time of onset of symptoms to diagnosis of 21 weeks. In many patients, the presentation mimicked that of chronic otitis media, delaying diagnosis. Histological subtype was embryonal in 13 patients and alveolar in 1. All patients underwent multimodality treatment. The 5-year disease-free survival rate was 81%. Regional post-treatment morbidity included chronic aural discharge (6/14), facial palsy (8/14), growth disturbance (4/14) and maxillo-facial deformity occurring in four children. From the results, we conclude that these patients should usually present to an ENT surgeon who should keep the diagnosis in mind when dealing with children with chronic otitis media as early diagnosis with referral to a specialist multidisciplinary team will optimize the chance of survival. Discharge, hearing loss and aural polyp, although commonly because of chronic otitis media, should prompt urgent investigation and biopsy, particularly if associated with facial palsy, lymphadenopathy or an obvious mass.

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

“…In 2 series, presentation was at approximately 4 years of age. 10 Notably, there are no reported cases of bilateral temporal bone RMS, in contrast to reports of bilateral temporal bone LCH. There is a slight male predominance in RMS patients.…”

“…There is a slight male predominance in RMS patients. 10 The presentation of RMS is most commonly mistaken for benign ear disease, resulting in a reported delay in diagnosis ranging from 4 to 78 weeks. 10 Chronic otitis media, polyps of the external auricular canal, and retroauricular masses are all common pre- sentations.…”

“…There are 4 distinct pathologic types of RMS: embryonal (most common in head and neck 10 ), alveolar, pleomorphic, and botryoid. Tumors can arise from striated muscle or from undifferentiated mesenchymal cells.…”

Clinical and Histopathological Study of Mass in Ear: A Study of Fifty Cases

“…Typically these patients present with post auricular non tender swelling with mass in external auditory canal which bleeds on touch. Symptoms include unilateral refractory otitis media with sero-sanguineous discharge and otalgia [15]. Exact site is difficult to determine [18].…”

“…They are the most common soft-tissue malignancy in the pediatric age group [13] . Rhabdomyosarcomas of the middle ear and mastoid are the most common malignant aural neoplasm, although they account for less than 10% of all cases of head and neck Rhabdomyosarcomas [14,15].…”