engleski

Our experience in the treatment and follow-up of family members with MEN IIA syndrome

After 25-year follow up in family members with MEN IIA syndrome, we evaluated the effects of early treat¬ ment. During this period, we identified 25 relatives. Eleven of them underwent total thyroidectomy ; in 8 patients histopathologic reports showed medullary thy¬ roid carcinoma, and in 3 young patients (aged 23-27 yrs) C-cell hyperplasia. The mean age of all relatives at the time of operation was 25.1 (16-41 yrs). Three relatives underwent bilateral adrenalectomy for pheochromocy-toma. All patients who underwent surgical intervention after 1982 (since when serum calcitonin level has been measured) had high serum calcitonin concentrations, either baseline or provoked (pentagastrin, alcohol). All these patients were positive on RET mutation testing. In 2 relatives moderately elevated calcitonin levels were measured postoperatively, without evidence for met-astates. In 4 relatives screening for pheochromocytoma was performed. No parathyroid adenoma/carcinoma was found in any of the family members. RET mutation test¬ ing was performed in 15 relatives in 1989 at the Insti¬ tute of Cancer Research, Sutton Surrey, UK. Since 1994, RET mutation testing in another 15 relatives was per¬ formed at Department of Molecular Medicine, Ruder Boskovic Insititute in Zagreb. The youngest relatives (born 1993-2007) underwent RET mutation analysis. Test results were positive in two (born in 1988 and 2000), and baseline calcitonin level was elevated in one of these subjects. The parents have not yet made decision on surgical intervention recommended by us. Our results are mostly consistent with other reports on the treat¬ ment and follow up of relatives with MEN IIA syndrome.

MEN IIA syndrome ; follow-up

Abstract of the Scientific Syposium with International Participation "40 Years of Nuclear Medicine in Osijek", November 15 - 17, 2007. Osijek, Croatia.