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Tumors of C Cells

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Atlas of Endocrine Pathology

Part of the book series: Atlas of Anatomic Pathology ((AAP))

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Abstract

Medullary thyroid carcinoma (MTC) comprises 5–10 % of thyroid carcinomas. About 25 % of cases are familial; thus, patients are offered RET mutation analysis. MTC occurs in multiple endocrine neoplasia 2A (MEN2A), MEN2B, and familial MTC (FMTC), autosomal dominant disorders due to RET mutations. MEN2A and FMTC are associated with RET mutations of codons 609, 611, 618, 620, and 634. In FMTC, mutations of codons 768, 790, 791, 804, 844, and 891 are associated with less aggressive disease and a later onset. Most MEN2B RET mutations are at codon 918 (>95 %) or 883 (2–3 %). MEN2A is associated with MTC, pheochromocytoma, and parathyroid disease. MEN2B is associated with MTC, pheochromocytoma, mucocutaneous neuromas, ganglioneuromatosis, and marfanoid habitus. MTC usually occurs before pheochromocytoma in MEN2A and MEN2B, and preoperative calcitonin levels may help identify MTC and prevent a hypertensive crisis during surgery.

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Authors and Affiliations

  1. Mayo Clinic College of Medicine, Rochester, MN, USA

    Lori A. Erickson MD

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  1. Lori A. Erickson MD
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Erickson, L.A. (2014). Tumors of C Cells. In: Atlas of Endocrine Pathology. Atlas of Anatomic Pathology. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-0443-3_11

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  • DOI: https://doi.org/10.1007/978-1-4939-0443-3_11

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4939-0442-6

  • Online ISBN: 978-1-4939-0443-3

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