Abstract
OBJECTIVES: The rarity of temporal bone squamous cell carcinoma (TBSCC) precludes a clear understanding of the disease and approach to its management. This review provides general background on the disease and discusses the current and emerging oncologic and rehabilitative management options. DATA SOURCES: PubMed literature review. METHODS: A review of the current literature was conducted to assess and collate up-to-date information regarding TBSCC management. RESULTS: TBSCC is a rare and aggressive disease arising in the ear canal, temporal bone, or extratemporal sites. Prior radiation, chronic ear disease, or habitual ear picking may contribute to primary disease development. Because the symptoms of TBSCC and benign otologic disease are similar, TBSCC diagnosis may be delayed, allowing the tumor time to spread throughout the anatomically intricate temporal bone. The extent of the disease is determined based on imaging and is usually staged with the Pittsburgh Staging System. Temporal bone resection with parotidectomy and neck dissection is the current standard of care. Survival is generally good for early disease and poor for advanced disease, but chemotherapy is emerging as a promising treatment option. Auditory rehabilitation with osseointegrated hearing aids is recommended at initial oncologic resection. CONCLUSIONS: The knowledge of and outcomes for TBSCC have improved with time, but because of the aggressive nature of the disease and the anatomic intricacy of the temporal bone, TBSCC treatment is complex and should be delivered by a multidisciplinary team. Inter-institutional collaboration may accelerate research for this rare disease. LEVEL OF EVIDENCE: 5.