Present status of prophylactic thyroidectomy in pediatric multiple endocrine neoplasia 2

A nationwide survey in Japan 1997-2017

Thyroid Committee of the Japanese Society for Pediatric Endocrinology

Research output: Contribution to journalReview article

Abstract

In Japan, prophylactic thyroidectomy involves out-of-pocket expense. The American Thyroid Association (ATA) recommends prophylactic thyroidectomy for medullary thyroid carcinoma (MTC) during early childhood in patients with multiple endocrine neoplasia type 2 (MEN2). The ATA reports a high frequency of postoperative complications in childhood, which also influenced the delay of prophylactic thyroidectomy in Japan. This retrospective study of multiple medical centers in Japan included individuals aged <20 years diagnosed with germline RET mutations between 1997 and 2017. The onset and onset possibility were defined based on confirmed lesions or calcitonin levels. The definition of risk and prophylactic thyroidectomy were based on the ATA 2015 revised guideline. Twenty-one patients with MEN2 were enrolled (highest risk, n = 5; high risk, n = 5; and moderate risk, n = 11). The cumulative incidence of the onset/onset possibility reached 50% at 5 and 8 years and 100% at 9 years and 17 years in high-and moderate-risk patients, respectively. Of 7 patients with MEN2A, 71% underwent prophylactic thyroidectomy. Only one 5-year-old patient (C634Y) had increased serum calcitonin level after prophylactic thyroidectomy in the MEN2A group. The only permanent complication, which did not occur in patients who underwent total thyroidectomy alone, was hypoparathyroidism (33% of patients). This permanent complication occurred with clinically developed MTC. No permanent postoperative complications occurred in patients aged 5-6 years. Prophylactic thyroidectomy reduces recurrence and postoperative complications in pediatric patients with MEN2. Early thyroidectomy based on only calcitonin level could possibly reduce thyroidectomy delay.

Original languageEnglish
Pages (from-to)585-595
Number of pages11
JournalJournal of Pediatric Endocrinology and Metabolism
Volume32
Issue number6
DOIs
Publication statusPublished - 2019 Jun 1

Fingerprint

Multiple Endocrine Neoplasia
Thyroidectomy
Japan
Pediatrics
Multiple Endocrine Neoplasia Type 2a
Calcitonin
Thyroid Gland
Surveys and Questionnaires
Hypoparathyroidism
Germ-Line Mutation
Health Expenditures
Retrospective Studies
Guidelines

Keywords

  • central node dissection
  • medullary thyroid carcinoma
  • multiple endocrine neoplasia type 2
  • postoperative complications
  • prophylactic thyroidectomy
  • recurrence

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

Cite this

Present status of prophylactic thyroidectomy in pediatric multiple endocrine neoplasia 2 : A nationwide survey in Japan 1997-2017. / Thyroid Committee of the Japanese Society for Pediatric Endocrinology.

In: Journal of Pediatric Endocrinology and Metabolism, Vol. 32, No. 6, 01.06.2019, p. 585-595.

Research output: Contribution to journalReview article

@article{8841ff8c45904ea38664dbfab233f55e,
title = "Present status of prophylactic thyroidectomy in pediatric multiple endocrine neoplasia 2: A nationwide survey in Japan 1997-2017",
abstract = "In Japan, prophylactic thyroidectomy involves out-of-pocket expense. The American Thyroid Association (ATA) recommends prophylactic thyroidectomy for medullary thyroid carcinoma (MTC) during early childhood in patients with multiple endocrine neoplasia type 2 (MEN2). The ATA reports a high frequency of postoperative complications in childhood, which also influenced the delay of prophylactic thyroidectomy in Japan. This retrospective study of multiple medical centers in Japan included individuals aged <20 years diagnosed with germline RET mutations between 1997 and 2017. The onset and onset possibility were defined based on confirmed lesions or calcitonin levels. The definition of risk and prophylactic thyroidectomy were based on the ATA 2015 revised guideline. Twenty-one patients with MEN2 were enrolled (highest risk, n = 5; high risk, n = 5; and moderate risk, n = 11). The cumulative incidence of the onset/onset possibility reached 50{\%} at 5 and 8 years and 100{\%} at 9 years and 17 years in high-and moderate-risk patients, respectively. Of 7 patients with MEN2A, 71{\%} underwent prophylactic thyroidectomy. Only one 5-year-old patient (C634Y) had increased serum calcitonin level after prophylactic thyroidectomy in the MEN2A group. The only permanent complication, which did not occur in patients who underwent total thyroidectomy alone, was hypoparathyroidism (33{\%} of patients). This permanent complication occurred with clinically developed MTC. No permanent postoperative complications occurred in patients aged 5-6 years. Prophylactic thyroidectomy reduces recurrence and postoperative complications in pediatric patients with MEN2. Early thyroidectomy based on only calcitonin level could possibly reduce thyroidectomy delay.",
keywords = "central node dissection, medullary thyroid carcinoma, multiple endocrine neoplasia type 2, postoperative complications, prophylactic thyroidectomy, recurrence",
author = "{Thyroid Committee of the Japanese Society for Pediatric Endocrinology} and Rie Matsushita and Keisuke Nagasaki and Tadayuki Ayabe and Yoko Miyoshi and Saori Kinjo and Hidenori Haruna and Kenji Ihara and Tomonobu Hasegawa and Shinobu Ida and Keiichi Ozono and Kanshi Minamitani and Tsutomu Ogata and Yasuko Fujisawa and Takashi Hamajima and Yuki Abe and Mika Inoue and Makiko Tachibana and Takuo Kubota and Noriyuki Namba and Masatsune Itoh and Yukihiro Hasegawa and Aya Shimada and Reiko Horikawa and Chikahiko Numakura and Ikuma Fujiwara and Hiroyo Mabe and Takeshi Usui and Satoshi Narumi and Tamotsu Kobayashi and Koichi Saito and Yuhki Arai and Kazuhiko Jinno and Yasutsugu Chinen and Mahoko Furujo and Atsushi Yoden and Emiri Kaji and Sotaro Mushiake and Tomoaki Taguchi and Akihiro Sakurai and Shinichi Suzuki and Shinya Uchino and Youko Miyoshi and Saori Kinjyo",
year = "2019",
month = "6",
day = "1",
doi = "10.1515/jpem-2018-0444",
language = "English",
volume = "32",
pages = "585--595",
journal = "Journal of Pediatric Endocrinology and Metabolism",
issn = "0334-018X",
publisher = "Walter de Gruyter GmbH & Co. KG",
number = "6",

}

TY - JOUR

T1 - Present status of prophylactic thyroidectomy in pediatric multiple endocrine neoplasia 2

T2 - A nationwide survey in Japan 1997-2017

AU - Thyroid Committee of the Japanese Society for Pediatric Endocrinology

AU - Matsushita, Rie

AU - Nagasaki, Keisuke

AU - Ayabe, Tadayuki

AU - Miyoshi, Yoko

AU - Kinjo, Saori

AU - Haruna, Hidenori

AU - Ihara, Kenji

AU - Hasegawa, Tomonobu

AU - Ida, Shinobu

AU - Ozono, Keiichi

AU - Minamitani, Kanshi

AU - Ogata, Tsutomu

AU - Fujisawa, Yasuko

AU - Hamajima, Takashi

AU - Abe, Yuki

AU - Inoue, Mika

AU - Tachibana, Makiko

AU - Kubota, Takuo

AU - Namba, Noriyuki

AU - Itoh, Masatsune

AU - Hasegawa, Yukihiro

AU - Shimada, Aya

AU - Horikawa, Reiko

AU - Numakura, Chikahiko

AU - Fujiwara, Ikuma

AU - Mabe, Hiroyo

AU - Usui, Takeshi

AU - Narumi, Satoshi

AU - Kobayashi, Tamotsu

AU - Saito, Koichi

AU - Arai, Yuhki

AU - Jinno, Kazuhiko

AU - Chinen, Yasutsugu

AU - Furujo, Mahoko

AU - Yoden, Atsushi

AU - Kaji, Emiri

AU - Mushiake, Sotaro

AU - Taguchi, Tomoaki

AU - Sakurai, Akihiro

AU - Suzuki, Shinichi

AU - Uchino, Shinya

AU - Miyoshi, Youko

AU - Kinjyo, Saori

PY - 2019/6/1

Y1 - 2019/6/1

N2 - In Japan, prophylactic thyroidectomy involves out-of-pocket expense. The American Thyroid Association (ATA) recommends prophylactic thyroidectomy for medullary thyroid carcinoma (MTC) during early childhood in patients with multiple endocrine neoplasia type 2 (MEN2). The ATA reports a high frequency of postoperative complications in childhood, which also influenced the delay of prophylactic thyroidectomy in Japan. This retrospective study of multiple medical centers in Japan included individuals aged <20 years diagnosed with germline RET mutations between 1997 and 2017. The onset and onset possibility were defined based on confirmed lesions or calcitonin levels. The definition of risk and prophylactic thyroidectomy were based on the ATA 2015 revised guideline. Twenty-one patients with MEN2 were enrolled (highest risk, n = 5; high risk, n = 5; and moderate risk, n = 11). The cumulative incidence of the onset/onset possibility reached 50% at 5 and 8 years and 100% at 9 years and 17 years in high-and moderate-risk patients, respectively. Of 7 patients with MEN2A, 71% underwent prophylactic thyroidectomy. Only one 5-year-old patient (C634Y) had increased serum calcitonin level after prophylactic thyroidectomy in the MEN2A group. The only permanent complication, which did not occur in patients who underwent total thyroidectomy alone, was hypoparathyroidism (33% of patients). This permanent complication occurred with clinically developed MTC. No permanent postoperative complications occurred in patients aged 5-6 years. Prophylactic thyroidectomy reduces recurrence and postoperative complications in pediatric patients with MEN2. Early thyroidectomy based on only calcitonin level could possibly reduce thyroidectomy delay.

AB - In Japan, prophylactic thyroidectomy involves out-of-pocket expense. The American Thyroid Association (ATA) recommends prophylactic thyroidectomy for medullary thyroid carcinoma (MTC) during early childhood in patients with multiple endocrine neoplasia type 2 (MEN2). The ATA reports a high frequency of postoperative complications in childhood, which also influenced the delay of prophylactic thyroidectomy in Japan. This retrospective study of multiple medical centers in Japan included individuals aged <20 years diagnosed with germline RET mutations between 1997 and 2017. The onset and onset possibility were defined based on confirmed lesions or calcitonin levels. The definition of risk and prophylactic thyroidectomy were based on the ATA 2015 revised guideline. Twenty-one patients with MEN2 were enrolled (highest risk, n = 5; high risk, n = 5; and moderate risk, n = 11). The cumulative incidence of the onset/onset possibility reached 50% at 5 and 8 years and 100% at 9 years and 17 years in high-and moderate-risk patients, respectively. Of 7 patients with MEN2A, 71% underwent prophylactic thyroidectomy. Only one 5-year-old patient (C634Y) had increased serum calcitonin level after prophylactic thyroidectomy in the MEN2A group. The only permanent complication, which did not occur in patients who underwent total thyroidectomy alone, was hypoparathyroidism (33% of patients). This permanent complication occurred with clinically developed MTC. No permanent postoperative complications occurred in patients aged 5-6 years. Prophylactic thyroidectomy reduces recurrence and postoperative complications in pediatric patients with MEN2. Early thyroidectomy based on only calcitonin level could possibly reduce thyroidectomy delay.

KW - central node dissection

KW - medullary thyroid carcinoma

KW - multiple endocrine neoplasia type 2

KW - postoperative complications

KW - prophylactic thyroidectomy

KW - recurrence

UR - http://www.scopus.com/inward/record.url?scp=85067172637&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85067172637&partnerID=8YFLogxK

U2 - 10.1515/jpem-2018-0444

DO - 10.1515/jpem-2018-0444

M3 - Review article

VL - 32

SP - 585

EP - 595

JO - Journal of Pediatric Endocrinology and Metabolism

JF - Journal of Pediatric Endocrinology and Metabolism

SN - 0334-018X

IS - 6

ER -