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Minimal Invasive Resection of Large Retrosternal Thyroid Goiter
Published on: September 20, 2024
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.
Abstract:
There is no evidence-based guidance on the extent of prophylactic neck surgery for second-generation multiple endocrine neoplasia type 2B (MEN 2B), a newly emerging entity in the molecular era. In this investigation of MEN 2B children who inherited the M918T RET germline mutation from a phenotypically affected MEN 2B parent, 6 MEN 2B children (4 girls and 2 boys) from 5 MEN 2B parents (4 mothers and 1 father) were identified. None of the 6 second-generation MEN 2B children who had preoperative calcitonin serum levels between 2 and 105 pg/mL and underwent prophylactic total thyroidectomy before the age of 4 years after receiving a positive RET gene test harbored node metastases. There was no recurrent laryngeal nerve palsy or postoperative hypoparathyroidism. Within the limitations of this study, total thyroidectomy alone is adequate therapy for second-generation MEN 2B children aged 1-4 years old with preoperative calcitonin serum levels ≤100 pg/mL.
Insights
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.
- 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.
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