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Prophylactic thyroidectomies in MEN2 syndrome: Management and outcomes
Virginie Bussières1, Shreyas Roy2, Johnny Deladoey3
1Faculty of Medicine, University of Montreal, Montreal, Quebec, Canada.
Journal of Pediatric Surgery
|January 17, 2018
Summary
Prophylactic thyroidectomy in children with multiple endocrine neoplasia type 2 (MEN2) syndrome is effective. Nine of 21 pediatric patients had microcarcinoma, with no permanent complications, underscoring the need for early surgical intervention.
Area of Science:
- Pediatric Endocrinology
- Surgical Oncology
- Genetics
Background:
- Multiple Endocrine Neoplasia type 2 (MEN2) is a hereditary condition predisposing individuals to medullary thyroid carcinoma (MTC).
- Prophylactic thyroidectomy is recommended for children with MEN2 to prevent MTC development.
- Evaluating outcomes in academic settings is crucial for refining surgical protocols.
Purpose of the Study:
- To assess the results of prophylactic thyroidectomies in pediatric patients diagnosed with MEN2 syndrome.
- To identify the incidence of MTC or precursor lesions in asymptomatic children undergoing preventive surgery.
Main Methods:
- A retrospective chart review was conducted on patients under 18 years old who underwent prophylactic thyroidectomy for MEN2 between 2006 and 2015.
- Patient data, including genetic mutations, pathological findings, and surgical outcomes, were analyzed.
Main Results:
- Twenty-one pediatric patients with MEN2 (19 with MEN2A) underwent prophylactic thyroidectomy; all were asymptomatic pre-operatively.
- Nine patients (43%) had MTC microcarcinoma, with all lesions <6mm; none had lymph node involvement.
- No permanent surgical complications were observed, though transient hypocalcemia occurred in six patients.
Conclusions:
- Prophylactic thyroidectomy in children with MEN2 is safe and effective in detecting early-stage MTC.
- The findings emphasize the importance of comprehensive family history and timely surgical intervention for familial thyroid cancer syndromes.
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