Robert Bruce MacIntosh1, Prasanna-Kumar Shivapuja2, Michael Brady Krzemien3, Michael Lee4. 1. Clinical Professor, Department of Oral and Maxillofacial Surgery, University of Detroit Mercy School of Dentistry, Detroit, MI. Electronic address: Mac999@bfomfs.com. 2. Orthodontic Consultant, Department of Oral and Maxillofacial Surgery, St. John Health System, Detroit, MI. 3. Chief Resident, Oral and Maxillofacial Surgery, St John Health System, Detroit, MI. 4. Senior Student, University of Detroit Mercy School of Dentistry, Detroit, MI.
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.
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: Medard F M van den Broek; Ester B G Rijks; Peter G J Nikkels; Victorien M Wolters; Robert J J van Es; Hanneke M van Santen; Bernadette P M van Nesselrooij; Menno R Vriens; Rachel S van Leeuwaarde; Gerlof D Valk; Annemarie A Verrijn Stuart Journal: Endocrine Date: 2021-01-21 Impact factor: 3.633