Multiple endocrine neoplasia type 2B: maxillofacial significance in 5 cases.

Abstract

PURPOSE:

This paper intends to review for the oral and maxillofacial surgery community the MEN abnormalities generally, to emphasize the maxillofacial abnormalities peculiar to the MEN2B variant particularly, and to demonstrate the importance of early recognition of its most important component, medullary thyroid carcinoma.

PATIENTS AND METHODS:

The findings in five individuals with confirmed diagnoses of MEN2B are arranged to demonstrate the various manifestations of the disorder, and together represent the largest single group in the oral and maxillofacial surgery literature to date.

RESULTS:

Tabulation of the patients' individual findings demonstrates the variants and severity of the disorder, discloses findings not previously emphasized in the literature, and stresses the significance of early recognition of medullary thyroid carcinoma.

CONCLUSION:

The evidence in this study group suggests that the oral mucosal lesions are more commonly neurofibromas than neuromas as previously reported, that the characteristic dental central diastemas may occur independent of tongue size or the presence of oral soft tissue lesions, and that the characteristic apertognathia potentially requires surgical correction.

• Authors: Robert Bruce MacIntosh, Prasanna-Kumar Shivapuja, Michael Brady Krzemien, Michael Lee
• Journal: Journal of oral and maxillofacial surgery : official journal of the American Association of Oral and Maxillofacial Surgeons (J Oral Maxillofac Surg) Vol. 72 Issue 12 Pg. 2498.e1-17 (Dec 2014) ISSN: 1531-5053 [Electronic] United States
• PMID: 25454714 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright © 2014 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

Topics

• Adult
• Child
• Face (pathology)
• Female
• Humans
• Male
• Maxilla (pathology)
• Multiple Endocrine Neoplasia Type 2b (pathology)
• Young Adult