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Multiple endocrine neoplasia syndrome type IIb: a case report
1Department of Child Dental Health, Glasgow Dental Hospital and School NHS Trust, Scotland.
International Journal of Paediatric Dentistry
|April 29, 1998
Summary
Multiple endocrine neoplasia type IIb (MEN IIb) is a rare syndrome with distinctive oral symptoms. Early identification through oral examination is crucial for timely diagnosis and management of MEN IIb.
Area of Science:
- Endocrinology
- Genetics
- Oral Medicine
Background:
- Multiple endocrine neoplasia type IIb (MEN IIb) is a rare genetic disorder.
- It is characterized by neoplastic changes in endocrine glands and distinctive oro-facial manifestations.
Observation:
- A pediatric patient presented with oral mucosal tags, premature permanent teeth eruption, malocclusion, and facial asymmetry.
- Initial biopsies suggested MEN IIb or neurofibromatosis, with genetic testing confirming MEN IIb.
Findings:
- The patient exhibited progressive oral lesions, including modular tongue lesions and lip enlargement, over a 9-year follow-up.
- No endocrine tumors have developed to date, highlighting a potential for delayed or absent tumorigenesis in some cases.
Implications:
- This case underscores the critical role of thorough oral mucosa examination in diagnosing rare genetic syndromes like MEN IIb.
- Regular follow-up of oral abnormalities is essential for early detection and management of potential complications associated with MEN IIb.