Results and follow-up in eleven MEN 2A gene carriers after prophylactic thyroidectomy

Experimental and Clinical Endocrinology & Diabetes : Official Journal, German Society of Endocrinology [and] German Diabetes Association
K Frank-RaueF Raue

Abstract

In 11 MEN 2A gene carriers prophylactic thyroidectomy was carried out between the age of 4 to 17 years. All gene carriers had pathological basal and/or pentagastrin stimulated serum calcitonin levels. On histological examination in five patients C-cell hyperplasia (CCH) and in 6 patients medullary microcarcinoma (< 1 cm, mostly multifocally) was shown. No patient had lymph node involvement. There was no recurrent laryngeal nerve damage in these 11 patients and no permanent hypoparathyroidism. After prophylactic thyroidectomy all 11 patients were cured, they showed normal basal and pentagastrin stimulated calcitonin levels. In the follow-up (mean 19 months) under thyroxine substitution therapy in 9 of these 11 patients TSH was at least at one examination out of the normal range. In 2 patients TSH was suppressed, in 1 of these FT3 was slightly elevated. In 7 patients TSH was elevated between 4.9 to 147 microU/ml. The application of genetic testing is save concerning diagnostic accuracy. If prophylactic thyroidectomy is done at early ages cure rate is 100%. Despite this encouraging results thyroxine substitution therapy in the follow-up carries some problems concerning optimal dosage and non-compliance. Therefore TSH has to be est...Continue Reading

Citations

Nov 20, 2003·European Journal of Surgical Oncology : the Journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology·A BoérO Esik
Jan 1, 2006·Hereditary Cancer in Clinical Practice·Jan Willem de GrootJohn Tm Plukker
Sep 29, 2001·Journal of Infusion Nursing : the Official Publication of the Infusion Nurses Society·E Giarelli

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