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]
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
The two major types of multiple endocrine neoplasia type 1 and type 2, are often confused because they have similar names. These types are distinguished by the genes involved, the hormones that are affected, and their characteristic signs and symptoms. They are also very different in their options for cancer prevention and treatment. Multiple endocrine neoplasia type 2 can be further divided into three subtypes: type 2A, type 2B, and familial medullary thyroid carcinoma (FMTC). These subtypes differ in their characteristic signs and symptoms and risk of specific tumors, but are relatively consistent within any one family.
MEN IIa is characterized by the presence of Medullary thyroid carcinoma, pheochromocytoma, and parathyroid hyperplasia or tumor.
MEN IIb is characterized by the presence of pheochromocytoma, mucocutaneous neuroma, and medullary thyroid cancer.[1]
| 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 cancer without pheochromocytoma or parathyroid hyperplasia | Medullary thyroid cancer, pheochromocytoma and mucosal neuromas or intestinal ganglioneuromas, marfanoid habitus | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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.