Temporal bone malignancies (TBM) are a rare but distinct group of conditions accounting for less than 0.2% of head and neck cancers and have an estimated annual incidence of less than 6 in 1 million. Squamous cell carcinoma (SCC) accounts for the majority of these malignancies, which is reflected in the literature since there are 21 published case series on temporal bone SCC and only case reports for non-squamous malignancies. TBMSCC has a poor prognosis in part due to their locally aggressive nature and complex anatomy. Progress in treatment remains slow given the rarity of this condition. Additionally, two main issues remain: 1. Lack of prognostic factors; 2. Multiple treatment strategies with little evidence on their effectiveness.

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