Abstract
Medullary thyroid cancer (MTC) accounts for 5% of all thyroid cancers. MTC originates from the parafollicular of the thyroid, which derive from the neural crest. In children, more than 95% cases of MTC are inherited as part of the multiple endocrine neoplasia syndromes (MEN2A and MEN2B) or familial MTC. MEN2B only represents 5% of MEN2 cases and is associated with a mutation in the M918T of the RET oncogene. MEN2B is characterized by specific phenotypic features, including a marfanoid habit, a prominent jaw, ocular abnormalities, ganglioneuromas and mucosal neuromas.
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