Middle ear adenocarcinoma is a very rare, locally invasive neoplasm assumed
to arise from the middle ear mucosa. Although endolymphatic sac tumor (agg
ressive papillary middle ear tumor) and jugulotympanic paraganglioma may sh
ow brain invasion, intracranial extension of histologically confirmed middl
e ear adenocarcinoma has not been previously reported. The authors describe
a 53-year-old man who suffered from otalgia and tinnitus for more than 10
years and from neurological deficits for 1 year due to a large temporal bon
e tumor that invaded the temporal lobe. A combined neurosurgical and otolar
yngological resection was performed. Pathological analysis revealed a low-g
rade adenocarcinoma of a mixed epithelial-neuroendocrine phenotype, which s
howed a close histological similarity to, and topographical relationship wi
th, middle ear epithelium. The authors conclude that middle ear adenocarcin
oma belongs to the spectrum of extracranial tumors that have possible local
extension to the brain.