Desmoplastic Fibroma of the Temporal Bone.

A 13-year-old male patient presented with a 9-month history of right aural fullness, progressive hearing loss, and pulsatile tinnitus. On examination, he had a stenotic, slit-like external auditory canal preventing visualization of the tympanic membrane. The patient reported a history of transient facial paresis. An audiogram showed a maximal conductive loss on the affected side. Facial nerve electroneuronography revealed normal facial nerve function. CT imaging revealed a large fibro-osseous lesion involving the entire lateral aspect of the right temporal bone, narrowing both the vertical fallopian canal and the lateral external auditory canal. There was no involvement of the otic capsule or internal auditory canal. MRI revealed an enhancing mass involving the lateral and superior aspects of the right temporal bone adjacent to the temporal lobe. Finally, a technetium nuclear medicine bone scan showed increased uptake in the area of interest. The patient was taken to the operating room for an open biopsy. Intraoperatively, the mass appeared densely fibrotic with irregular appearance of the involved bone. Final pathology revealed a paucity of spindle cells in a background of abundant collagenous tissue, consistent with desmoplastic fibroma (Fig. 1). He then underwent a complete resection, which was performed via infratemporal fossa approach with overclosure of the ear canal and simultaneous placement of a bone-anchored hearing aid. He has done well since that time with no evidence of recurrence 18 months after primary resection.