Squamous cell carcinoma of the temporal bone arising 43 years after fenestration procedure

Squamous cell carcinoma originating in the middle ear cleft is a rare tumor that tends to be diagnosed late in its course. The presenting symptoms mimic inflammatory disease of the middle ear space, and hence biopsy is often delayed. Herein we present a case of squamous cell carcinoma of the temporal bone arising 43 years after fenestration procedure. Operative findings were significant for complete dissolution of the tegmen tympani, tegmen mastoideum, and posterior fossa dura plate with dural exposure and sigmoid sinus exposure. Tumor was noted to invade the labyrinth at the horizontal semicircular canal. This case report highlights the aggressiveness of this particular tumor in a previously exenterated mastoid cavity and its proclivity to penetrate the otic capsule via pre-formed pathways. The treatment of choice is aggressive surgical resection followed by radiotherapy.