Aggressive papillary middle ear tumors: A report of two cases with review of the literature

ADENOMATOUS TUMORS OF the middle ear are rare, with only approximately 100 cases reported. A distinct subclass of this tumor demonstrates microscopic papillary architecture and has a propensity to erode the petrous bone and extend intracranially. The term “aggressive papillary middle ear tumor” has recently been proposed to describe this more invasive type of middle ear tumor. Thirty-seven cases of aggressive papillary middle ear tumors have been reported. We present two additional cases and discuss the clinical, radiologic, histologic, and operative features of this locally aggressive neoplasm. The tumor affects adults of both sexes. The clinical prodrome is prolonged. Presenting signs and symptoms most often relate to the involvement of cranial nerves V-VIII. Imaging studies reveal large, enhancing, destructive tumors with a generous vascular supply. Intraoperatively, the tumors are bloody, fibrous, and adherent to surrounding structures. Various surgical approaches in combination or in series may be used. Preoperative embolization may be helpful. The role of adjunctive radiation is unclear. Aggressive papillary middle ear tumors are histologically benign tumors with clinically destructive behavior. However, it appears that aggressive surgical management affords prolonged survival with minimal worsening of cranial nerve deficits.