Temporal bone findings in multiple endocrine neoplasia type 2B

Abstract

To our knowledge, present case is the first published report of temporal bone findings in multiple endocrine neoplasia type 2B (MEN-2B). We describe a 43-year-old Japanese man with medullary thyroid carcinoma (MTC), pheochromocytoma, mucosal neuroma and a Marfanoid body habitus. The collateral adrenal 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 detected in the liver, lungs, kidneys, pancreas and cervical lymph nodes. Recurrent pheochromocytoma was present in the right kidney. Mucosal neuromas were found in the tongue, gastrointestinal tract and vesical nerve plexus. The following histopathological findings were seen in both temporal bones: metastatic MTC was found as well as neuromas and the cochlear aqueduct was widely patent.

Multiple endocrine neoplasia type 2B with hyperparathyroidism: A case report and literature review

“…However, many patients have lost the opportunity for radical surgery owing to the extremely strong invasiveness of MTC which would have distant metastasis earlier. There were studies exploring drugs for the treatment of this disease [23,24] , among which targeted drugs were the most. In a randomized controlled study, Vandetanib had an overall control rate of 68% for inoperable MTC [25] .…”

Temporal bone metastasis from thyroid

“…[3,4] Patients with temporal bone metastasis may remain asymptomatic or present with a variety of complaints, such as, sudden hearing loss, dizziness, tinnitus, facial palsy, otalgia, swelling behind the ear, bleeding from the ear, and even features of increased intracranial tension. Any patient presenting with facial palsy, sudden sensory neural hearing loss, and periauricular swelling should raise suspicion of a metastatic temporal bone tumor.…”