Temporal bone findings in multiple endocrine neoplasia type 2B

To our knowledge, present case is the first published report of temporal bo ne findings in multiple endocrine neoplasia type 2B (MEN-2B). We describe a 43-year-old Japanese man with medullary thyroid carcinoma (MTC), pheochrom ocytoma, mucosal neuroma and a Marfanoid body habitus, The collateral adren al tumors and MTC were removed surgically. However, 14 years after surgery, the MTC and pheochromocytoma recurred and the patient died of intracranial hemorrhage due to hypertension, During the autopsy, metastatic MTC was det ected in the liver, lungs, kidneys, pancreas and cervical lymph nodes. Recu rrent pheochromocytoma was present in the right kidney. Mucosal neuromas we re found in the tongue, gastrointestinal tract and vesical nerve plexus. Th e following histopathological findings were seen in both temporal bones: me tastatic MTC was found as well as neuromas and the cochlear aqueduct was wi dely patent.