Management And Results Of Thyroidectomies In Pediatric Patients With Men 2 Syndrome
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In brief
In brief
This retrospective study of 30 pediatric patients with MEN 2 syndrome demonstrates that prophylactic thyroidectomy performed by experienced pediatric surgeons is safe and effective in preventing medullary thyroid carcinoma. Early genetic testing and family counseling are critical for timely intervention, with most patients undergoing surgery around age 7 based on RET mutation risk stratification.
Written by the GCMD Library team from the article.
Abstract
Aim of the study
To evaluate the outcome of prophylactic thyroidectomies (PT) in patients with MEN 2 syndrome in a tertiary center.
Methods
A retrospective study was designed, including all patients with MEN 2 syndrome who underwent PT between 2000 and 2019. Demographics, gene mutation, postoperative complications and histopathological findings were registered.
Main results
30 patients were included (29 MEN 2A and 1 MEN 2B) with a median age at surgery time of 7.0 ± 3.2 years. Familiar history was present in all but 3 patients. A therapeutic thyroidectomy was performed in 2 patients due to evidence of medullary thyroid carcinoma (MTC, both were late diagnosis), and in the other 28 cases, a PT was performed. 8 patients had a RET mutation ranked as Moderate Risk (American Thyroid Association): median age at surgery was 7.2 ± 4.2 years, and histological findings were C-cell hyperplasia (n = 6) and no alterations (n = 2). 16 patients had a high risk mutation; median age at surgery time was 6.9 ± 2.8 years and histological findings were normal thyroid gland (n = 1), C Cell Hyperplasia (n = 8), microcarcinoma (n = 6), and MTC (n = 1). The mean hospital stay was 1.4 ± 0.68 days. No intraoperative complications or recurrent laryngeal nerve injuries were registered. 7 patients presented a transient hypoparathyroidism and 1 patient had permanent hypoparathyroidism.
Conclusions
Early PT in patients with MEN 2 syndrome is a safe procedure when performed by an experienced team of Pediatric Surgeons and with a multidisciplinary approach. Early genetic analysis and familial counselling is essential to prevent the development of a MTC.