Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma

doi:10.1089/thy.2014.0335

Review

. 2015 Jun;25(6):567-610.

doi: 10.1089/thy.2014.0335.

Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma

Samuel A Wells Jr¹, Sylvia L Asa², Henning Dralle³, Rossella Elisei⁴, Douglas B Evans⁵, Robert F Gagel⁶, Nancy Lee⁷, Andreas Machens³, Jeffrey F Moley⁸, Furio Pacini⁹, Friedhelm Raue¹⁰, Karin Frank-Raue¹⁰, Bruce Robinson¹¹, M Sara Rosenthal¹², Massimo Santoro¹³, Martin Schlumberger¹⁴, Manisha Shah¹⁵, Steven G Waguespack⁶; American Thyroid Association Guidelines Task Force on Medullary Thyroid Carcinoma

Affiliations

¹ 1Genetics Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland.
² 2Department of Pathology, University Health Network, and Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada.
³ 3Department of General, Visceral, and Vascular Surgery, University Hospital, University of Halle-Wittenberg, Halle/Saale, Germany.
⁴ 4Department of Endocrinology, University of Pisa, Pisa, Italy.
⁵ 5Department of Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin.
⁶ 6Department of Endocrine Neoplasia and Hormonal Disorders, Division of Internal Medicine, The University of Texas MD Anderson Cancer Center, Houston, Texas.
⁷ 7Department of Radiation Oncology, Memorial Sloan-Kettering Cancer Center, New York, New York.
⁸ 8Department of Surgery, Washington University School of Medicine, St. Louis, Missouri.
⁹ 9Section of Endocrinology and Metabolism, Department of Internal Medicine, Endocrinology and Metabolism and Biochemistry, University of Siena, Policlinico Santa Maria alle Scotte, Siena, Italy.
¹⁰ 10Endocrine Practice, Moleculargenetic Laboratory, Medical Faculty, University of Heidelberg, Heidelberg, Germany.
¹¹ 11University of Sydney School of Medicine, Sydney, New South Wales, Australia.
¹² 12Departments of Internal Medicine, Pediatrics and Behavioral Science, University of Kentucky, Lexington, Kentucky.
¹³ 13Dipartimento di Medicina Molecolare e Biotecnologie Mediche, Universita' di Napoli "Federico II," Napoli, Italy.
¹⁴ 14Institut Gustave Roussy, Service de Medecine Nucleaire, Université of Paris-Sud, Villejuif, France.
¹⁵ 15Division of Medical Oncology, Department of Internal Medicine, The Ohio State University, Columbus, Ohio.

PMID: 25810047
PMCID: PMC4490627
DOI: 10.1089/thy.2014.0335

Review

Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma

Samuel A Wells Jr et al. Thyroid. 2015 Jun.

. 2015 Jun;25(6):567-610.

doi: 10.1089/thy.2014.0335.

Authors

Affiliations

¹ 1Genetics Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland.
² 2Department of Pathology, University Health Network, and Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada.
³ 3Department of General, Visceral, and Vascular Surgery, University Hospital, University of Halle-Wittenberg, Halle/Saale, Germany.
⁴ 4Department of Endocrinology, University of Pisa, Pisa, Italy.
⁵ 5Department of Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin.
⁶ 6Department of Endocrine Neoplasia and Hormonal Disorders, Division of Internal Medicine, The University of Texas MD Anderson Cancer Center, Houston, Texas.
⁷ 7Department of Radiation Oncology, Memorial Sloan-Kettering Cancer Center, New York, New York.
⁸ 8Department of Surgery, Washington University School of Medicine, St. Louis, Missouri.
⁹ 9Section of Endocrinology and Metabolism, Department of Internal Medicine, Endocrinology and Metabolism and Biochemistry, University of Siena, Policlinico Santa Maria alle Scotte, Siena, Italy.
¹⁰ 10Endocrine Practice, Moleculargenetic Laboratory, Medical Faculty, University of Heidelberg, Heidelberg, Germany.
¹¹ 11University of Sydney School of Medicine, Sydney, New South Wales, Australia.
¹² 12Departments of Internal Medicine, Pediatrics and Behavioral Science, University of Kentucky, Lexington, Kentucky.
¹³ 13Dipartimento di Medicina Molecolare e Biotecnologie Mediche, Universita' di Napoli "Federico II," Napoli, Italy.
¹⁴ 14Institut Gustave Roussy, Service de Medecine Nucleaire, Université of Paris-Sud, Villejuif, France.
¹⁵ 15Division of Medical Oncology, Department of Internal Medicine, The Ohio State University, Columbus, Ohio.

PMID: 25810047
PMCID: PMC4490627
DOI: 10.1089/thy.2014.0335

Abstract

Introduction: The American Thyroid Association appointed a Task Force of experts to revise the original Medullary Thyroid Carcinoma: Management Guidelines of the American Thyroid Association.

Methods: The Task Force identified relevant articles using a systematic PubMed search, supplemented with additional published materials, and then created evidence-based recommendations, which were set in categories using criteria adapted from the United States Preventive Services Task Force Agency for Healthcare Research and Quality. The original guidelines provided abundant source material and an excellent organizational structure that served as the basis for the current revised document.

Results: The revised guidelines are focused primarily on the diagnosis and treatment of patients with sporadic medullary thyroid carcinoma (MTC) and hereditary MTC.

Conclusions: The Task Force developed 67 evidence-based recommendations to assist clinicians in the care of patients with MTC. The Task Force considers the recommendations to represent current, rational, and optimal medical practice.

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Figures

<b>FIG. 1.</b> — **FIG. 1.**
Management of patients with a thyroid nodule and histological diagnosis of medullary thyroid carcinoma. ADX, adrenalectomy; Ctn, calcitonin; CEA, carcinoembryonic antigen; EBRT, external beam radiotherapy; FNA, fine-needle aspiration; HPTH, hyperparathyroidism; LND, lymph node dissection; MTC, medullary thyroid carcinoma; M, metastatic MTC; PHEO, pheochromocytoma; *RET*, REarranged during Transfection; TKI, tyrosine kinase inhibitor; TTX, total thyroidectomy; US, ultrasound.

<b>FIG. 2.</b> — **FIG. 2.**
Management of patients with a *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.

<b>FIG. 3.</b> — **FIG. 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.

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Comment in

RET Y791F Variant Does Not Increase the Risk for Medullary Thyroid Carcinoma.
Toledo RA, Maciel RM, Erlic Z, Lourenço DM Jr, Cerutti JM, Eng C, Neumann HP, Toledo SP. Toledo RA, et al. Thyroid. 2015 Aug;25(8):973-4. doi: 10.1089/thy.2015.0168. Epub 2015 Jun 12. Thyroid. 2015. PMID: 25950813 No abstract available.

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References

1. Cancer of the Thyroid Invasive: Trends in SEER Incidence and U.S. Mortality Using the Joinpoint Regression Program, 1975–2011(SEER) Stat version 8.1.2 Rate Session. (Access the SEER 18 database at www.seer.cancer.gov). Incidence - SEER 18 Regs Research Data+Hurricane Katrina Impacted Louisiana Cases, Nov 2012 Sub (2000–2010)<Katrina/Rita Population Adjustment>- Linked To County Attributes - Total U.S., 1969–2011 Counties, National Cancer Institute, DCCPS, Surveillance Research Program, Surveillance Systems Branch, released April 2013, based on the November 2012 submission
1. Brandi ML, Gagel RF, Angeli A, Bilezikian JP, Beck-Peccoz P, Bordi C, Conte-Devolx B, Falchetti A, Gheri RG, Libroia A, Lips CJ, Lombardi G, Mannelli M, Pacini F, Ponder BA, Raue F, Skogseid B, Tamburrano G, Thakker RV, Thompson NW, Tomassetti P, Tonelli F, Wells SA, Jr, Marx SJ. 2001. Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab 86:5658–5671 - PubMed
1. Tuttle RM, Ball DW, Byrd D, Daniels GH, Dilawari RA, Doherty GM, Duh QY, Ehya H, Farrar WB, Haddad RI, Kandeel F, Kloos RT, Kopp P, Lamonica DM, Loree TR, Lydiatt WM, McCaffrey J, Olson JA, Jr, Parks L, Ridge JA, Shah JP, Sherman SI, Sturgeon C, Waguespack SG, Wang TN, Wirth LJ. 2010. Medullary carcinoma. J Natl Compr Canc Netw 8:512–530 - PubMed
1. Chen H, Sippel RS, O'Dorisio MS, Vinik AI, Lloyd RV, Pacak K. 2010. The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer. Pancreas 39:775–783 - PMC - PubMed
1. Kloos RT, Eng C, Evans DB, Francis GL, Gagel RF, Gharib H, Moley JF, Pacini F, Ringel MD, Schlumberger M, Wells SA., Jr 2009. Medullary thyroid cancer: management guidelines ofthe American Thyroid Association. Thyroid 19:565–612 - PubMed

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[1] Cancer of the Thyroid Invasive: Trends in SEER Incidence and U.S. Mortality Using the Joinpoint Regression Program, 1975–2011(SEER) Stat version 8.1.2 Rate Session. (Access the SEER 18 database at www.seer.cancer.gov). Incidence - SEER 18 Regs Research Data+Hurricane Katrina Impacted Louisiana Cases, Nov 2012 Sub (2000–2010)<Katrina/Rita Population Adjustment>- Linked To County Attributes - Total U.S., 1969–2011 Counties, National Cancer Institute, DCCPS, Surveillance Research Program, Surveillance Systems Branch, released April 2013, based on the November 2012 submission

[2] Cancer of the Thyroid Invasive: Trends in SEER Incidence and U.S. Mortality Using the Joinpoint Regression Program, 1975–2011(SEER) Stat version 8.1.2 Rate Session. (Access the SEER 18 database at www.seer.cancer.gov). Incidence - SEER 18 Regs Research Data+Hurricane Katrina Impacted Louisiana Cases, Nov 2012 Sub (2000–2010)<Katrina/Rita Population Adjustment>- Linked To County Attributes - Total U.S., 1969–2011 Counties, National Cancer Institute, DCCPS, Surveillance Research Program, Surveillance Systems Branch, released April 2013, based on the November 2012 submission

[3] Brandi ML, Gagel RF, Angeli A, Bilezikian JP, Beck-Peccoz P, Bordi C, Conte-Devolx B, Falchetti A, Gheri RG, Libroia A, Lips CJ, Lombardi G, Mannelli M, Pacini F, Ponder BA, Raue F, Skogseid B, Tamburrano G, Thakker RV, Thompson NW, Tomassetti P, Tonelli F, Wells SA, Jr, Marx SJ. 2001. Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab 86:5658–5671 - PubMed

[4] Brandi ML, Gagel RF, Angeli A, Bilezikian JP, Beck-Peccoz P, Bordi C, Conte-Devolx B, Falchetti A, Gheri RG, Libroia A, Lips CJ, Lombardi G, Mannelli M, Pacini F, Ponder BA, Raue F, Skogseid B, Tamburrano G, Thakker RV, Thompson NW, Tomassetti P, Tonelli F, Wells SA, Jr, Marx SJ. 2001. Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab 86:5658–5671 - PubMed

[5] Tuttle RM, Ball DW, Byrd D, Daniels GH, Dilawari RA, Doherty GM, Duh QY, Ehya H, Farrar WB, Haddad RI, Kandeel F, Kloos RT, Kopp P, Lamonica DM, Loree TR, Lydiatt WM, McCaffrey J, Olson JA, Jr, Parks L, Ridge JA, Shah JP, Sherman SI, Sturgeon C, Waguespack SG, Wang TN, Wirth LJ. 2010. Medullary carcinoma. J Natl Compr Canc Netw 8:512–530 - PubMed

[6] Tuttle RM, Ball DW, Byrd D, Daniels GH, Dilawari RA, Doherty GM, Duh QY, Ehya H, Farrar WB, Haddad RI, Kandeel F, Kloos RT, Kopp P, Lamonica DM, Loree TR, Lydiatt WM, McCaffrey J, Olson JA, Jr, Parks L, Ridge JA, Shah JP, Sherman SI, Sturgeon C, Waguespack SG, Wang TN, Wirth LJ. 2010. Medullary carcinoma. J Natl Compr Canc Netw 8:512–530 - PubMed

[7] Chen H, Sippel RS, O'Dorisio MS, Vinik AI, Lloyd RV, Pacak K. 2010. The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer. Pancreas 39:775–783 - PMC - PubMed

[8] Chen H, Sippel RS, O'Dorisio MS, Vinik AI, Lloyd RV, Pacak K. 2010. The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer. Pancreas 39:775–783 - PMC - PubMed

[9] Kloos RT, Eng C, Evans DB, Francis GL, Gagel RF, Gharib H, Moley JF, Pacini F, Ringel MD, Schlumberger M, Wells SA., Jr 2009. Medullary thyroid cancer: management guidelines ofthe American Thyroid Association. Thyroid 19:565–612 - PubMed

[10] Kloos RT, Eng C, Evans DB, Francis GL, Gagel RF, Gharib H, Moley JF, Pacini F, Ringel MD, Schlumberger M, Wells SA., Jr 2009. Medullary thyroid cancer: management guidelines ofthe American Thyroid Association. Thyroid 19:565–612 - PubMed

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Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma

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Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma

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