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Early total thyroidectomy in children exposed to the risk of hereditary medullary cancer: report of 17 observations

L. Coupris*, R. Coutant**, N. Bouhours**, F. Gatelais**, V. Rohmer***, E. Eyssartier*, D. Geslin*, D. Weil*.

* Department of Visceral Pediatric Surgery, Pediatric Unit, CHU Angers

** Department of Pediatric Endocrinology, Pediatric Unit, CHU Angers

*** Department of Adult Endocrinology, CHU Angers, France



Introduction: Thyroid cancer accounts for 1% of the pediatric malignant tumours; 10% are hereditary thyroid medullary cancer (familial or NEM). This pathology has a dominant transmission, the description of the RET mutation reveals the patients with 100% risk exposure. The cancer can develop in youngsters, so that prevention means early total thyroidectomy.

Material and methods: Our 17 observations, 9 boys and 8 girls, examined in the pediatric department, come from 11 families followed by the adult endocrinologists. All have the RET mutation. Seven of them belong to four families with NEM II A. The total thyroidectomy was performed at an average age of 77 months (28 to 133).

Results: The C cells hyperplasia was constant. Medullary micro-cancers were already present in 7 cases; the youngest was 28 months old (5 times in only one lobe; 2 times in the two lobes). There were no metastatic lesions in the surgical specimen. 2/17 will present a transitory hypoparathyroidism and a 1/17 unilateral recurrent paralysis with diagnosed secondary. The average follow-up is 79 months (2-140). A long term monitoring is necessary. discussion: The total thyroidectomy is not particularly technically difficulty in pediatric patients. The inherent question is the age of the child at surgery so that the treatment can be preventive and not only early curative. The indications provided by the literature show regular lowering of the recommended age. A modulation can be done according to the type of the genetic mutation and its “aggressiveness”. Association of NEM II B with “Hirschsprung’s disease” is an indication for total thyroidectomy in the first months of life.

Conclusion: The genetic screening of the subjects at the risk for familial thyroid medullary cancer reveals more frequent indications for early total thyroidectomy in young children. The pediatric surgeons must prepare to face this particular activity field.


Key words: thyroid, hereditary medullar cancer, child