Int J Stomatol

Inter J Stomatol ›› 2018, Vol. 45 ›› Issue (1): 36-41.doi: 10.7518/gjkq.2018.01.007

• Original Articles • Previous Articles     Next Articles

Research progress on multiple endocrine neoplasia type 2

Zhang Meihua1, Hu Ying2, Sun Zheng3   

  1. 1. Dept. of Stomatology, The Fourth Affiliated Hospital of Inner Mongolia Medical University, Baotou 014030, China
    2. Institute of Dental Research, Beijing Stomatological Hospital & School of Stomatology, Capital Medical University, Beijing 100050, China
    3. Dept. of Oral Medicine, Beijing Stomatological Hospital & School of Stomatology, Capital Medical University, Beijing 100050, China
  • Received:2017-04-07 Revised:2017-10-12 Online:2018-01-01 Published:2018-01-01

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Abstract:

Multiple endocrine neoplasia type 2 (MEN2) is a rare autosomal dominant disease. This condition can be classified into three types, including MEN2A, MEN2B, and familial medullary thyroid carcinoma (MTC). Primary clinical manifestations of this type of neoplasia include the following: MTC, pheochromocytoma, hyperparathyroidism, Hirschsprung’s disease, bone and facial dysplasia, and multiple nerve fibroma of oral mucosa. MTC contributes mainly to early morbidity and high metastasis rate. Patients with MEN2B are prompted to consult with a dentist primarily due to occurrence of multiple mucosa neuroma. Missense mutations in RET gene result in MEN2. RET is located on chromosome 10. Mutations in genotypes of RET genes correlate with phenotype mutations, including age of onset and degree of malignancy. Clinical experiments determined operation time and plan according to genotype. Gene mutations induces changes in RET gene activities, resulting in changes in intra and extracellular signaling pathways. This paper summarizes research progress on clinical and molecular basis, mechanism of RET gene structure and function changes, and MEN2 diagnosis and treatments.

Key words: multiple endocrine neoplasia type 2, medullary thyroid carcinoma, pheochromocytoma, Marfan signs, multiple mucosa neuroma

CLC Number: 

  • R739.8

TrendMD: 
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