BSPED2011 Poster Presentations (1) (84 abstracts)
Prophylactic thyroidectomy in children with MEN2 in the United Kingdom
Francesco Paolo Prete 1 , Clare Morkane 1 , J Watkinson 2 , N Shaw 2 , S Alvi 3 , R Squire 3 , B Harrison 4 , J Wales 4 , P Clayton 5 , P Morrison 6 , D Carson 6 , C Brain 1 , P Hindmarsh 1 , M Dattani 1 , H Spoudeas 1 , C Buchanan 7 , A Albanese 8 , R Amin 9 , A Piero 1 & T Kurzawinski 1
1University College Hospital, Great Ormond Street Hospital, London, UK; 2Birmingham Children’s Hospital, Birmingham, UK; 3Leeds General Infirmary, Leeds, UK; 4Sheffield Children’s Hospital, Sheffield, UK; 5Royal Manchester Children’s Hospital, Manchester, UK; 6Royal Belfast Hospital for Sick Children, Belfast, UK; 7King’s College Hospital, London, UK; 8S. George’s Hospital, London, UK; 9S. Bartholomew’s Hospital, London, UK.
Introduction: Timing, extent, complications rate and long term results of paediatric prophylactic thyroidectomy (pPT) for MEN2 in the UK are unknown.
Methods: All UK centers performing pPT were invited to participate in the study.Data were obtained from notes and hospitals electronic databases.
Results: Fifty-one children (27 males) were included.All had genetic test at the mean age of 5 years (median 3, range 0.25–15), confirming 45 MEN2 and 6 FMTC. Mean baseline preoperative calcitonin was 27.34 (median 12, range 0–290). Ten patients underwent preoperative pentagastrin stimulation test. Fourty-seven had surgery (47 total thyroidectomise, of which three had central and one lateral nodes dissection) and four are awaiting operation. Surgery was performed at a mean age of 7 years (median 5.47, range 0.74–20.88). Postoperative calcium was low in 65% of children but only eight required prolonged calcium treatment. Respiratory distress with aspiration pneumonia developed in the only patient who had bilateral neck dissection. Histology was available in 42 cases (17 medullary cancers, 22 C-cell hyperplasia and 3 normal thyroid glands). In seven patients lymph nodes were examined at histology and all were found negative. Median follow-up was 35.95 months (range 0, 2–142).There were no relapses. At year 1–5 postoperative calcitonin was detectable in 4/26 (4 out of 26 tested), 7/15, 8/15, 2/4 and 2/5 children respectively.At year 2–5 it was elevated in one, two, two and one children respectively.
Conclusion: pPT is a safe procedure,with a low rate of postoperative complications. Commonest operation was total thyroidectomy without lymphadenectomy. Timing of surgery is guided by RET mutation and basal calcitonin levels rather than pentagastrin stimulation. The relevance of detectable and marginally elevated levels of postoperative calcitonin in uncertain and only long term follow up will give us insight into its significance.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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