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Prophylactic thyroidectomy in children with multiple endocrine neoplasia type 2

Prete, FP; Abdel-Aziz, T; Morkane, C; Brain, C; Kurzawinski, TR; MEN2 in Children UK Collaborative Group; (2018) Prophylactic thyroidectomy in children with multiple endocrine neoplasia type 2. British Journal of Surgery , 105 (10) pp. 1319-1327. 10.1002/bjs.10856. Green open access

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Abstract

Background In patients with multiple endocrine neoplasia type 2 (MEN2) syndrome, genetic testing offers early diagnosis, stratifies the risk of developing medullary thyroid cancer (MTC) and informs the timing of thyroidectomy. The efficacy of treatment, which depends on timely and safe surgery, is not well established. Methods This was a retrospective review of diagnostic and clinicopathological outcomes of prophylactic thyroidectomy in children with MEN2 between 1995 and 2013 in the UK. American Thyroid Association (ATA) 2009 guidelines were used as a benchmark for adequate treatment. Results Seventy‐nine children from 16 centres underwent total thyroidectomy. Thirty‐eight patients (48 per cent) underwent genetic testing and 36 (46 per cent) had an operation performed above the age recommended by the ATA 2009 guidelines; pathology showed MTC in 30 patients (38 per cent). Late surgery, above‐normal preoperative calcitonin level and MTC on pathology correlated with late genetic testing. Twenty‐five children had lymphadenectomy; these patients had more parathyroid glands excised (mean difference 0·61, 95 per cent c.i. 0·24 to 0·98; P = 0·001), and were more likely to have hypocalcaemia requiring medication (relative risk (RR) 3·12, 95 per cent c.i. 1·54 to 6·32; P = 0·002) and permanent hypoparathyroidism (RR 3·24, 1·29 to 8·11; P = 0·010) compared with those who underwent total thyroidectomy alone. Age did not influence the development of complications. Conclusion Late genetic testing may preclude age‐appropriate surgery, increasing the risk of operating when MTC has already developed. Early genetic testing and age‐appropriate surgery may help avoid unnecessary lymphadenectomy and improve outcomes.

Type: Article
Title: Prophylactic thyroidectomy in children with multiple endocrine neoplasia type 2
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1002/bjs.10856
Publisher version: https://doi.org/10.1002/bjs.10856
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: MEN2, medullary thyroid cancer, prophylactic thyroidectomy, pediatric, precision medicine
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Surgery and Interventional Sci
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Genetics and Genomic Medicine Dept
URI: https://discovery.ucl.ac.uk/id/eprint/10080735
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