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Licensed Unlicensed Requires Authentication Published by De Gruyter October 1, 2021

Medullary thyroid carcinoma in children: current state of the art and future perspectives

  • Andreas Kiriakopoulos , Anastasia Dimopoulou ORCID logo EMAIL logo , Constantinos Nastos , Dimitra Dimopoulou , Konstantina Dimopoulou , Evangelos Menenakos and Nikolaos Zavras


Medullary thyroid carcinoma (MTC) is a distinct type of malignant thyroid tumor in cell origin, biological behavior, and natural history. It accounts for 1.6% of all thyroid cancers and presents either sporadically or as a hereditary disease, the latter occurring as a part of multiple endocrine neoplasia (MEN) 2A and MEN2B syndromes or as a familial MTC disease with no other manifestations. The gene responsible for the hereditary form is the rearranged during transfection (RET) gene, a proto-oncogene located to human chromosome 10. Most pediatric MTC cases have been discovered after genetic testing investigations, leading to the concept of prophylactic surgery in presymptomatic patients. Therefore, the genetic status of the child, along with serum calcitonin levels and ultrasonographic findings, determine the appropriate age for prophylactic surgical intervention. Nevertheless, a diagnosis at an early stage of MTC warrants total thyroidectomy and central lymph node dissection with the addition of lateral/contralateral lymph node dissection depending on the tumor size, ultrasonographic evidence of neck disease, or calcitonin levels. Conversely, locally advanced/unresectable or metastatic MTC is primarily treated with multikinase inhibitors, while more specific RET inhibitors are being tested in clinical trials with promising results.

Corresponding author: Dr. Anastasia Dimopoulou, Department of Pediatric Surgery, University General Hospital “ATTIKON”, National and Kapodistrian University of Athens School of Medicine, 1 Rimini Street Str, 124 62, Athens, Greece , Phone: +0030 2105831299, Fax: +0030 2105832229, E-mail:

  1. Research funding: None declared.

  2. Author contributions: Kyriakopoulos A drafted the first manuscript. Dimopoulou A wrote and revised the final draft. Constantinos Nastos analyzed and interpreted the collected data. Dimopoulou D and Dimopoulou K performed the literature search and selected articles with supervision by Menenakos E. Zavras N conceived and designed the study. All the authors have accepted responsibility for the entire content of this submitted manuscript and approved submission.

  3. Competing interests: The funding organization(s) played no role in the study design; in the collection, analysis, and interpretation of data; in the writing of the report; or in the decision to submit the report for publication.


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Received: 2021-07-27
Accepted: 2021-09-20
Published Online: 2021-10-01
Published in Print: 2022-01-27

© 2021 Walter de Gruyter GmbH, Berlin/Boston

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