Advances in the management of MEN2: from improved surgical and medical treatment to novel kinase inhibitors

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   Figure 1

   The results of 50 consecutive patients (mean age 10 years; range 3–19 years) who had inherited a mutated RET allele and were treated by prophylactic thyroidectomy. All patients were evaluated 5–10 years after surgery (mean 7 years) by physical examination and measurement of serum calcitonin levels following the combined administration of calcium and pentagastrin. Basal and stimulated serum calcitonin levels were undetectable in 44 patients, and they were considered cured. Basal serum calcitonin levels were undetectable in 4 patients but they increased above the normal range following stimulation 4–9 years after thyroidectomy. In two patients, basal and stimulated serum calcitonin levels were above the normal range, one immediately after thyroidectomy and the other not until 3 years after thyroidectomy. Data from Skinner et al. (2005).
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   Figure 2

   Management of patients with an RET germline mutation detected on genetic screening. ATA, American Thyroid Association risk categories for aggressive medullary thyroid carcinoma (MTC) (HST, highest risk, H, high risk, MOD, moderate risk); Ctn, calcitonin; CEA, carcinoembryonic antigen; HPTH, hyperparathyroidism; PHEO, pheochromocytoma; RET, REarranged during Transfection; TTX, total thyroidectomy; US, ultrasound: Reproduced, with permission, from Wells et al. (2015). Copyright 2015 American Thyroid Association and Mary Ann Liebert Inc.
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   Figure 3

   Management of patients following thyroidectomy for persistent or recurrent medullary thyroid carcinoma. Ctn, calcitonin; CEA, carcinoembryonic antigen; EBRT, external beam radiotherapy; MTC, medullary thyroid carcinoma; TFTs, thyroid function tests; TSH, thyrotropin; TKI, tyrosine kinase inhibitor; TTX, total thyroidectomy; US, ultrasound. Reproduced, with permission, from Wells et al. (2015). Copyright 2015 American Thyroid Association and Mary Ann Liebert Inc.
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   Figure 4

   A waterfall plot showing reduction in size of metastatic MTC in children with MEN2B who received vanetanib. Reproduced from Fox et al. (2013), with permission from the American Association for Cancer Research.

• © 2018 Society for Endocrinology