Neural hyperplasia in maxillary bone of multiple endocrine neoplasia type 2B patient

Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics
Yu UsamiSatoru Toyosawa


Multiple endocrine neoplasia (MEN) type 2B is the rarest and most aggressive form of MEN syndrome. MEN 2B patients manifest characteristic oral and facial features besides the neural crest cell-derived tumors, including medullary carcinoma, pheochromocytoma, mucosal neuroma, and ganglioneuromatosis of the gut. We report a case of MEN 2B diagnosed on the basis of the warning signs of mucosal neuroma and multiple neural hyperplasias in the maxillary bone resected during orthognathic surgery. A subsequent systemic examination under the pathologic diagnosis of neural lesions revealed medullary thyroid carcinoma, megacolon, thickened corneal nerves, and RET gene mutation, thus verifying the diagnosis of MEN 2B. An immunohistochemical study revealed an increased number of unmyelinated Schwann cells in the hyperplastic nerves. We suggest that intraosseous neural hyperplasia is a specific finding of the MEN 2B syndrome in addition to the known oral and facial manifestations.


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Aug 31, 2014·Atlas of the Oral and Maxillofacial Surgery Clinics of North America·David M Shafer, Lisa Marie Di Pasquale
Dec 3, 2014·Journal of Oral and Maxillofacial Surgery : Official Journal of the American Association of Oral and Maxillofacial Surgeons·Robert Bruce MacIntoshMichael Lee
Jul 22, 2014·Special Care in Dentistry : Official Publication of the American Association of Hospital Dentists, the Academy of Dentistry for the Handicapped, and the American Society for Geriatric Dentistry·Liana Preto WebberMaria Cristina Munerato

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