title: Multiple endocrine neoplasia in an orthodontic patient: Interprofessional diagnostic and treatment implications.
abstract: BACKGROUND
  Multiple endocrine neoplasia, type 2B (MEN 2B), is an autosomal-dominant condition characterized by the development of multiple endocrine tumors. All affected people develop an aggressive form of medullary thyroid cancer (MTC). Without early prophylactic thyroidectomy, the prognosis for patients with MEN 2B is poor; the average age at death is 21 years.
  
  
  METHODS
  The authors present a case of a 16-year-old girl who had a diagnosis of MEN 2B and was treated successfully for metastatic MTC.
  
  
  CONCLUSIONS
  Given the striking orofacial manifestations of MEN 2B (marfanoid habitus; dolichocephaly; everted and thickened lips; mucosal neuromas on lips, tongue, buccal mucosa and eyelids), dental professionals are well positioned to recognize the disorder. Early identification of patients with the condition permits screening for preclinical thyroid disease, molecular genetic testing, counseling and lifesaving thyroid surgery.
authors.affiliation: Department of Orthodontics, University College London Hospitals NHS Trust (UCLHT) Eastman Dental Hospital, 256 Gray's Inn Road, London, WC1X 8LD, England. joannecollins1@nhs.net
authors.forename: Joanne M
authors.initials: JM
authors.lastname: Collins
authors.forename: Shahid I
authors.initials: SI
authors.lastname: Chaudhry
authors.forename: Daljit S
authors.initials: DS
authors.lastname: Gill
authors.forename: Stephen R
authors.initials: SR
authors.lastname: Porter
mesh_terms: D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000175:diagnosis; D002116:Calcitonin / Q000378:metabolism; D019066:Facies; D005260:Female; D006258:Head and Neck Neoplasms / Q000378:metabolism / Q000532:radiotherapy / Q000601:surgery; D006801:Humans; D008312:Malocclusion, Angle Class II / Q000150:complications*; D009062:Mouth Neoplasms / Q000175:diagnosis; D009377:Multiple Endocrine Neoplasia / Q000150:complications / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D020125:Mutation, Missense; D037981:Neck Dissection; D009463:Neuroma / Q000175:diagnosis; D010673:Pheochromocytoma / Q000175:diagnosis; D051096:Proto-Oncogene Proteins c-ret / Q000235:genetics*
chemical_list: D002116:Calcitonin; D051096:Proto-Oncogene Proteins c-ret