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Inherited Medullary Thyroid Carcinoma: Indications and Technique of Early Thyroidectomy

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Medullary Thyroid Cancer

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Abstract

Medullary thyroid carcinoma (MTC) occurring as part of the dominantly inherited multiple endocrine neoplasia (MEN) type 2 syndromes is a rare but notable endocrine disease. Since the discovery of the role of activating mutations in the RET proto-oncogene in MEN2A and MEN2B, considerable advances in management have occurred, and most of the asymptomatic RET carriers who have undergone early thyroidectomy can lead full lives unencumbered by the presence of metastatic MTC. Strong genotype–phenotype correlations have enabled the development of guidelines regarding the timing of thyroidectomy, and current approaches to decisions about the age of surgery for MEN2A incorporate data from ultrasonography and measurement of calcitonin in addition to the results of genotyping. MEN2B is associated with the most aggressive clinical presentation of MTC and thus requires earlier surgical intervention. To optimize care and to enable ongoing research, patients with hereditary MTC in the context of the MEN2 syndromes are optimally treated at tertiary centers with multidisciplinary expertise.

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Authors and Affiliations

  1. Department of Surgical Oncology, MD Anderson Cancer Center, The University of Texas, 1400 Pressler Street, Unit 1484, Houston, TX, 77030, USA

    Elizabeth G. Grubbs

  2. Department of Endocrine Neoplasia and Hormonal Disorders, MD Anderson Cancer Center, The University of Texas, 1400 Pressler Street, Unit 1461, Houston, TX, 77030, USA

    Steven G. Waguespack

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Correspondence to Steven G. Waguespack .

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  1. Associate Professor of Surgery; Chief, Section of Endocrine Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin, USA

    Tracy S. Wang

  2. Donald C. Ausman Family Foundation Professor in Surgery and Chair, Medical College of Wisconsin, Milwaukee, Wisconsin, USA

    Douglas B. Evans

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Grubbs, E.G., Waguespack, S.G. (2016). Inherited Medullary Thyroid Carcinoma: Indications and Technique of Early Thyroidectomy . In: Wang, T., Evans, D. (eds) Medullary Thyroid Cancer. Springer, Cham. https://doi.org/10.1007/978-3-319-39412-1_8

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