2019 Volume 65 Issue 2 Pages 99-104
Multiple endocrine neoplasia type 2B (MEN2B) is the rarest form of the disease and is known to cause multiple mucosal neuroma in the oral cavity, associated with medullary thyroid carcinoma (MTC) and pheochromocytoma (PC). We describe a 27-year-old man who was referred to our hospital after seeking medical attention of the oral cavity because of dyspnea. Immediately after his arrival, he had cardiopulmonary arrest and received life-saving treatment. On examination, the initial diagnosis was pheochromocytoma crisis attributed to PC. Further oral examinations were required for a suspected thyroid tumor to ascertain the likelihood of MEN2B. Extra-oral findings were bumpy lips, while intra-oral findings were multiple nodules on the tongue, lip mucosa, and maxillary gingiva. Since all results of the biopsy proved mucosal neuroma, the final diagnosis was MEN2B based on the fact that there was MTC. In many cases of MEN2B, neuroma of the oral mucosa often appears to multiply on visible surfaces before the actual onset of MTC, and PC and creates a characteristic complexion. To realize the early diagnosis and effective treatment of MEN2B, it is essential to be adequately aware of the relation between mucosal neuroma and MEN2B.
