Multiple Endocrine Neoplasia Type 2b

Similar to MEN2A, it is also caused by mutations of the MEN2 gene, also known as the RET proto-oncogene. Its clinical symptoms include medullary carcinoma (CARCINOMA, MEDULLARY) of THYROID GLAND and PHEOCHROMOCYTOMA of ADRENAL MEDULLA (50%). Unlike MEN2a, MEN2b does not involve PARATHYROID NEOPLASMS. It can be distinguished from MEN2A by its neural abnormalities such as mucosal NEUROMAS on EYELIDS; LIP; and TONGUE, and ganglioneuromatosis of GASTROINTESTINAL TRACT leading to MEGACOLON. It is an autosomal dominant inherited disease.

Also Known As:

MEN 3; MEN2b; MEA 2b; MEA IIb; MEN III; MEN IIb; Mucosal Neuroma Syndrome; Multiple Endocrine Neoplasia, Type 2b; Multiple Endocrine Neoplasia, Type IIb; Multiple Endocrine Neoplasms Type 2b; Neuromata, Mucosal, With Endocrine Tumors; Wagenmann-Froboese Syndrome; Mucosal Neuroma Syndromes; Neuroma Syndrome, Mucosal; Syndrome, Wagenmann-Froboese; Wagenmann Froboese Syndrome; MEN 2b; Neoplasia, Multiple Endocrine Type 2b; Neoplasms, Multiple Endocrine Type 2b

Neoplasms: 1361496
- Hereditary Neoplastic Syndromes: 115
  - Multiple Endocrine Neoplasia: 508
    - Multiple Endocrine Neoplasia Type 2b: 498
- Multiple Primary Neoplasms: 24
  - Multiple Endocrine Neoplasia: 508
    - Multiple Endocrine Neoplasia Type 2b: 498
- Neoplasms by Site: 4
  - Endocrine Gland Neoplasms: 135
    - Multiple Endocrine Neoplasia: 508
      - Multiple Endocrine Neoplasia Type 2b: 498
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 960
- Inborn Genetic Diseases: 12342
  - Hereditary Neoplastic Syndromes: 115
    - Multiple Endocrine Neoplasia: 508
      - Multiple Endocrine Neoplasia Type 2b: 498
Endocrine System Diseases: 996
- Endocrine Gland Neoplasms: 135
  - Multiple Endocrine Neoplasia: 508
    - Multiple Endocrine Neoplasia Type 2b: 498

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Disease

Drug/Important Bio-Agent (IBA)