Medullary thyroid carcinoma (MTC) arises from the parafollicular C cells of the thyroid and accounts for 3%-5% of thyroid cancers. MTC is more aggressive than most other thyroid cancer subtypes and thus accounts for a higher percentage of thyroid cancer deaths. Sporadic MTC accounts for 75% of cases; the remaining 25% arise in inherited multiple endocrine neoplasia type 2 (MEN2) A and B, autosomal-dominant syndromes caused by germline-activating mutations in the RET proto-oncogene. A majority of sporadic MTCs also harbor activating RET mutations, though these are somatic rather than germline mutations. Germline RET testing is necessary for all new cases to identify patients with MEN2 A and B. In addition, next-generation sequencing is playing an increasingly important role in MTCs, allowing for identification of tumors harboring activating somatic RET mutations.
How familiar are you with RET mutations in MTC? Test your knowledge with this quick quiz.
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Cite this: Lori Wirth. Fast Five Quiz: Medullary Thyroid Cancer and RET Mutation - Medscape - Aug 31, 2021.