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Related Experiment Video

Updated: Nov 29, 2025

Minimal Invasive Resection of Large Retrosternal Thyroid Goiter
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Prophylactic neck surgery for second-generation multiple endocrine neoplasia type 2B.

Andreas Machens1, Kerstin Lorenz1, Frank Weber2

  • 1Medical Faculty, Department of Visceral, Vascular and Endocrine Surgery, Martin Luther University Halle-Wittenberg, Ernst-Grube-Str. 40, D-06097, Halle (Saale), Germany.

European Journal of Surgical Oncology : the Journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
|November 23, 2020
PubMed
Summary

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Prophylactic total thyroidectomy is adequate for children with second-generation Multiple Endocrine Neoplasia type 2B (MEN 2B) carrying the M918T RET mutation. This approach, performed before age 4, prevented node metastases without complications.

Area of Science:

  • Endocrinology
  • Genetics
  • Pediatric Surgery

Background:

  • Multiple Endocrine Neoplasia type 2B (MEN 2B) is a rare genetic disorder characterized by medullary thyroid carcinoma, pheochromocytoma, and ganglioneuromas.
  • The M918T RET germline mutation is the primary driver of MEN 2B.
  • Optimal surgical management for pediatric second-generation MEN 2B remains undefined.

Purpose of the Study:

  • To evaluate the effectiveness of prophylactic total thyroidectomy in children with second-generation MEN 2B.
  • To assess the incidence of lymph node metastases and surgical complications in these patients.

Main Methods:

  • Retrospective analysis of 6 second-generation MEN 2B children from 5 affected parents.
  • All children carried the M918T RET mutation and underwent prophylactic total thyroidectomy before age 4.
Keywords:
Medullary thyroid cancerMultiple endocrine neoplasia type 2BPrecision medicineProphylactic ThyroidectomySerum Calcitonin

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  • Preoperative calcitonin levels ranged from 2 to 105 pg/mL.

    • Main Results:

    • None of the 6 children who underwent prophylactic total thyroidectomy before age 4 showed lymph node metastases.
    • No cases of recurrent laryngeal nerve palsy or postoperative hypoparathyroidism were observed.
    • Calcitonin levels in the studied children were between 2 and 105 pg/mL.

    Conclusions:

    • Total thyroidectomy alone appears adequate for second-generation MEN 2B children aged 1-4 years with preoperative calcitonin levels ≤100 pg/mL.
    • This surgical approach is associated with low risk of complications.
    • Further research is needed to confirm these findings in larger cohorts.