Rhabdomyosarcoma of the temporal bone in children – a systematic review

BACKGROUND: Rhabdomyosarcoma of the temporal bone and middle ear is a rare but aggressive pediatric malignancy that often mimics chronic otitis media, resulting in delayed diagnosis and advanced disease at presentation. OBJECTIVE: To summarize the epidemiology, clinical presentation, imaging characteristics, staging, management strategies, and outcomes of pediatric temporal bone rhabdomyosarcoma. METHODS: A review was conducted using Embase (Ovid), MEDLINE (PubMed), Cochrane Library, and ScienceDirect databases. Articles published between 2000 and 2025 were screened. Inclusion criteria comprised histopathologically confirmed rhabdomyosarcoma involving the temporal bone or middle ear in patients aged 0-18 years, with documented imaging and clinical data. RESULTS: Thirty-five studies, including 68 pediatric patients, were analyzed. The most common presenting symptoms were persistent otorrhea (73%), otalgia (42%), hearing loss, and aural polyps. Facial nerve palsy occurred in 69% of cases. Imaging demonstrated an osteolytic soft-tissue mass involving the middle ear and mastoid, often with skull base or intracranial extension. Chemotherapy combined with radiotherapy was the standard treatment approach. CONCLUSION: Temporal bone rhabdomyosarcoma in children should be considered in the differential diagnosis of persistent otologic symptoms, particularly when associated with facial nerve palsy or a poor response to standard therapy. Early imaging and biopsy are essential for early diagnosis and improved outcomes.