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

Abstract

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.

References

Jun 1, 1992·Archives of Internal Medicine·H F VasenC J Lips
Apr 1, 1992·International Journal of Oral and Maxillofacial Surgery·M E SchenbergP C Reade
Oct 1, 1990·The British Journal of Oral & Maxillofacial Surgery·M OhishiH Tashiro
Jan 1, 1985·Annals of the New York Academy of Sciences·S H Yen, K L Fields
Sep 1, 1982·The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society·D DahlA Bignami
May 1, 1981·Oral Surgery, Oral Medicine, and Oral Pathology·A J CasinoD Wolk
Feb 1, 1981·American Journal of Ophthalmology·B SpectorS A Wells
Sep 1, 1996·Journal of Medical Genetics·P J Morrison, N C Nevin
Aug 3, 2000·European Archives of Oto-rhino-laryngology : Official Journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : Affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery·H FukayaI Ohtani
Sep 7, 2001·Cytokine & Growth Factor Reviews·M Takahashi
Oct 17, 2003·The New England Journal of Medicine·Andreas MachensUNKNOWN European Multiple Endocrine Neoplasia (EUROMEN) Study Group
Jan 27, 2009·Arquivos brasileiros de endocrinologia e metabologia·Cleber P CamachoRui M B Maciel
May 28, 2009·Thyroid : Official Journal of the American Thyroid Association·UNKNOWN American Thyroid Association Guidelines Task ForceSamuel A Wells
Jan 7, 2010·The Angle Orthodontist·Brent AccursoCarl M Allen
Jan 20, 2010·Molecular and Cellular Endocrinology·Karin Frank-RaueFriedhelm Raue
Jan 21, 2010·Familial Cancer·Friedhelm Raue, Karin Frank-Raue

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Citations

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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