Present status of prophylactic thyroidectomy in children with multiple endocrine neoplasia 2 in Japan: An overview article from a nationwide survey of the Japanese Society for Pediatric Endocrinology

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  • Matsushita Rie
    Thyroid Committee of the Japanese Society for Pediatric Endocrinology Division of Pediatrics, Kikugawa General Hospital Department of Pediatrics, Hamamatsu University School of Medicine
  • Nagasaki Keisuke
    Thyroid Committee of the Japanese Society for Pediatric Endocrinology Division of Pediatrics, Department of Homeostatic Regulation and Development, Niigata University Graduate School of Medical and Dental Sciences
  • Ayabe Tadayuki
    Thyroid Committee of the Japanese Society for Pediatric Endocrinology Yuusuzu clinic for children and youths
  • Miyoshi Yoko
    Thyroid Committee of the Japanese Society for Pediatric Endocrinology Department of Pediatrics, Osaka University Graduate School of Medicine Department of Health and Nutrition, Osaka Shoin Women’s University
  • Kinjo Saori
    Thyroid Committee of the Japanese Society for Pediatric Endocrinology Division of Pediatrics, Okinawa Chubu Hospital
  • Haruna Hidenori
    Thyroid Committee of the Japanese Society for Pediatric Endocrinology Department of Pediatrics and Adolescent Medicine, Juntendo University Graduate School of Medicine
  • Ihara Kenji
    Thyroid Committee of the Japanese Society for Pediatric Endocrinology Department of Pediatrics, Faculty of Medicine, Oita University
  • Hasegawa Tomonobu
    Thyroid Committee of the Japanese Society for Pediatric Endocrinology Department of Pediatrics, Keio University School of Medicine
  • Ida Shinobu
    Department of Gastroenterology and Endocrinology, Osaka Women’s and Children’s Hospital; and Pediatric Chronic Intestinal Pseudo-Obstruction (CIPO), Research Group for “Comprehensive Study and Seamless Guidelines” on Rare and Intractable Gastrointestinal Disease from Childhood, Supported by the Ministry of Health, Labor and Welfare of Japan
  • Ozono Keiichi
    Department of Pediatrics, Osaka University Graduate School of Medicine
  • Minamitani Kanshi
    Thyroid Committee of the Japanese Society for Pediatric Endocrinology Department of Pediatrics, Teikyo University Chiba Medical Center

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Other Title
  • 多発性内分泌腫瘍2型小児患者の甲状腺髄様癌と医療状況について～日本小児内分泌学会全国調査からみえた本邦における小児期予防的甲状腺全摘術／極早期摘出の現状～

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Abstract

<p>Multiple endocrine neoplasia type 2 (MEN2) is caused by germline mutations in the RET gene. Medullary thyroid carcinoma (MTC) with MEN2 has almost complete penetrance. MTC has a high frequency of postoperative recurrence. Hereditary MTC arises at a young age. Moreover, the risk of hereditary MTC increases with age.</p><p>Prophylactic thyroidectomy for MTC during early childhood in patients with MEN2 is recommended worldwide. Prophylactic thyroidectomy in patients with MEN2 is considered to be the best approach for avoiding recurrence; however, thyroidectomy during early childhood has a higher frequency of postoperative complications. Previous studies, including ours, reported that prophylactic thyroidectomy may decrease recurrence and postoperative complications in pediatric patients with MEN2. Prophylactic thyroidectomy is total thyroidectomy without lymphadenectomy when the lesion is considered to be limited to the thyroid. Therefore, prophylactic thyroidectomy has achieved a lower frequency of postoperative complications in early childhood.</p><p>In Japan, prophylactic thyroidectomy is not covered by the national health insurance system, which, in combination with the difficult balance between preventing recurrence and postoperative complications, results in most children not undergoing prophylactic resection. Hence, early calcitonin level-based thyroidectomy that is not based on age may reduce postoperative complications and improve the prognosis of pediatric patients with MEN2 in Japan due to large heterogeneity in the age of onset and aggressiveness of MTC.</p>

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