甲状腺髓样癌治疗的最新进展

周 　庆, 吴 耀禄
延安大学附属医院腺体血管外科

摘要

甲状腺髓样癌（MTC）是一种神经内分泌肿瘤，约占甲状腺恶性肿瘤的3%-5%，通常比分化型甲状腺癌更
具侵袭性。MTC虽然不常见，但其独特的特征与其他内分泌肿瘤有特殊关联。目前，手术切除仍然是治疗的主要手
段，尽管放疗和最新的靶向治疗已用于转移性疾病，然而辅助治疗的研究仍然有限。因此，本综述总结了MTC管理
的最新证据和指南，对该疾病的治疗提供更新的理论依据。

关键词

甲状腺髓样癌；治疗

参考

[1]Wells SA Jr, Asa SL, Dralle H, et al. Revised

American Thyroid Association guidelines for the management

of medullary thyroid carcinoma. Thyroid. 2015;25(6):567-610.

[2]Hadoux J, Pacini F, Tuttle RM, Schlumberger M.

Management of advanced medullary thyroid cancer. Lancet

Diabetes Endocrinol. 2016 Jan;4(1):64-71.

[3]Elisei R, Cosci B, Romei C, et al. Prognostic

significance of somatic RET oncogene mutations in sporadic

medullary thyroid cancer: a 10-year follow-up study. J Clin

Endocrinol Metab. 2008;93(3):682-687.

[4]Mulligan LM, Kwok JB, Healey CS, et al. Germ-line

mutations of the RET proto-oncogene in multiple endocrine

neoplasia type 2A. Nature. 1993;363(6428):458-460.

[5]Marsh DJ, Learoyd DL, Andrew SD, et al. Somatic

mutations in the RET proto-oncogene in sporadic medullary

thyroid carcinoma. Clin Endocrinol (Oxf). 1996;44(3):249-257.

[6]Agrawal N, Jiao Y, Sausen M, et al. Exomic sequencing

of medullary thyroid cancer reveals dominant and mutually

exclusive oncogenic mutations in RET and RAS. J Clin

Endocrinol Metab. 2013;98(2):E364-E369.

[7]Moura MM, Cavaco BM, Pinto AE, Leite V. High

prevalence of RAS mutations in RET-negative sporadic

medullary thyroid carcinomas. J Clin Endocrinol Metab.

2011;96(5):E863-E868.

[8]Boichard A, Croux L, Al Ghuzlan A, et al. Somatic

RAS mutations occur in a large proportion of sporadic

RET-negative medullary thyroid carcinomas and extend to

a previously unidentified exon. J Clin Endocrinol Metab.

2012;97(10):E2031-E2035.

[9]Machens A, Dralle H. Biomarker-based risk

stratification for previously untreated medullary thyroid cancer.

J Clin Endocrinol Metab. 2010;95(6):2655-2663.

[10]Konstantinidis A, Stang M, Roman SA, Sosa JA.

Surgical management of medullary thyroid carcinoma. Updates

Surg. 2017;69(2):151-160.

[11]Wells SA Jr, Asa SL, Dralle H, et al. Revised

American Thyroid Association guidelines for the management

of medullary thyroid carcinoma. Thyroid. 2015;25(6):567-610.

[12]Wells SA Jr, Robinson BG, Gagel RF, et al.

Vandetanib in patients with locally advanced or metastatic

medullary thyroid cancer: a randomized, double-blind phase

III trial [published correction appears in J Clin Oncol. 2013

Aug 20;31(24):3049]. J Clin Oncol. 2012;30(2):134-141.

[13]Elisei R, Schlumberger MJ, Müller SP, et al.

Cabozantinib in progressive medullary thyroid cancer

[published correction appears in J Clin Oncol. 2014 Jun

10;32(17):1864]. J Clin Oncol. 2013;31(29):3639-3646.

[14]Schlumberger M, Elisei R, Müller S, et al. Overall

survival analysis of EXAM, a phase III trial of cabozantinib in

patients with radiographically progressive medullary thyroid

carcinoma. Ann Oncol. 2017;28(11):2813-2819.

[15]Hińcza-Nowak K, Kowalik A, Walczyk A, et al.

Immune Profiling of Medullary Thyroid Cancer-An Opportunity

for Immunotherapy. Genes (Basel). 2021;12(10):1534.

Published 2021 Sep 28.

[16]Jayasinghe R, Basnayake O, Jayarajah U, Seneviratne

S. Management of medullary carcinoma of the thyroid: a review.

J Int Med Res. 2022;50(7):3000605221110698.

[17]Dash A, Chakraborty S, Pillai MR, Knapp FF Jr.

Peptide receptor radionuclide therapy: an overview. Cancer

Biother Radiopharm. 2015;30(2):47-71.

[18]Gubbi S, Koch CA, Klubo-Gwiezdzinska J. Peptide

Receptor Radionuclide Therapy in Thyroid Cancer. Front

Endocrinol (Lausanne). 2022;13:896287. Published 2022 May

30.

[19]Rowell NP. The role of external beam radiotherapy

in the management of medullary carcinoma of the thyroid: A

systematic review. Radiother Oncol. 2019;136:113-120.