Multiple endocrine neoplasia type 2 classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2] Sogand Goudarzi, MD [3]
Overview
Multiple endocrine neoplasia type 2 may be classified according to the types of tumor involved into 2 subtypes: multiple endocrine neoplasia type 2A and multiple endocrine neoplasia type 2B.
Classification
Multiple endocrine neoplasia type 2 (MEN2) can be further divided into three subtypes: type 2A, type 2B, and familial medullary thyroid carcinoma (FMTC).[1][2]
- These subtypes differ in their characteristic signs and symptoms and risk of specific tumors, but are relatively consistent within any one family.
- Multiple endocrine neoplasia type 2A is characterized by the presence of medullary thyroid carcinoma, pheochromocytoma, and parathyroid hyperplasia or tumor.
- Multiple endocrine neoplasia type 2B is characterized by the presence of pheochromocytoma, mucocutaneous neuroma, and medullary thyroid cancer.
- The following flowchart depicts the classification of multiple endocrine neoplasia type 2.
| Multiple endocrine neoplasia type 2 | |||||||||||||||||||||||||||||||||||||||||
| Multiple endocrine neoplasia type 2A | Multiple endocrine neoplasia type 2B | ||||||||||||||||||||||||||||||||||||||||
| Multiple endocrine neoplasia type 2A classical | Multiple endocrine neoplasia type 2A with cutaneous lichen amyloidosis | Multiple endocrine neoplasia type 2A with Hirschsprung disease | Familial medullary thyroid carcinoma without pheochromocytoma or parathyroid hyperplasia | Medullary thyroid cancer, pheochromocytoma,marfanoid habitus, and mucosal neuromas or intestinal ganglioneuromas | |||||||||||||||||||||||||||||||||||||
References
- ↑ Wells SA, Asa SL, Dralle H, Elisei R, Evans DB, Gagel RF; et al. (2015). "Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma". Thyroid. 25 (6): 567–610. doi:10.1089/thy.2014.0335. PMC 4490627. PMID 25810047.
- ↑ Hansford, J. R (2000). "Multiple endocrine neoplasia type 2 and RET: from neoplasia to neurogenesis". Journal of Medical Genetics. 37 (11): 817–827. doi:10.1136/jmg.37.11.817. ISSN 1468-6244.