Prophylactic Thyroidectomy in Pediatric A Reference Center Experience

LIBERTO D H; SANSÓ G,; BARONTINI M; ACHA O

Buenos Aires, Argentina

Congreso; VII Congresso f the Federation of Pediatric Surgical Association of South Cone of América- CIPESUR; 2007

CIPESUR

Thyroid carcinoma represents around 3% of al! pediatric malignancies, and medullai thyroid carcinoma (MTC) constitutes 2% to 5 % of them. 25% of MTC is hereditary, 20% as MultipI endocrine neoplasia (MEN) and 5% as Familiar medullary thyroid carcinoma (FMTC). MTC usually the first presenring tumor of MEN and is the most common cause of death. Prophylactic rot,d rhyroidectomy is now recommended after having confirmed RET mutarions in children of parenreni’ with MEN or FMTC. Aim: Rewiewed our experience to determine the incidence of MTC with respect to age at surgerl, molecular diagnosis and basal calcitonin levels. Methods: We studied 6 patients with prophylacric rhyroidectomy from 01/2005 to 12/201)11 in our Hospital. Wc recorded: age at molecular diagnosis, familiar history, symptoms, image ani molecular studies, basal and postestimulation calcitonin levels, operative finds, surgical morbility ami anaromoparhology resulrs. Resulta: Median age of the 6 patients (3 boys and 3 girls) included in this study was 7 years (ranp, 5-16 years). Familiar history was: 2 patients with mothers with MEN II A, 2 patienrs (rwice) whh father wirh MEN II B, and 2 others (causins) wirh mother or father wirh FMTC. Ah cases wci asympromaric, and thyroid ecography were normal. AlI parienta presented somaric mutarions 1 RET proro-oncogen. Calcironin preoperarory basal leveis was normal in all cases (pacienrs wiíii parenrs wirh MEN II B presenta superior normal levels). 1Q’e did flor use Pentagastrin srimulared rn Anaromoparology resulrs were: 3 pacienrs presented bilateral MTC wirh C cells hiperplasia (CCI II 2 presenred CCH (one wirh asociated rhyroidiris) and rhe lasr one prescnred normal hisrology. Tbci were no dearhs nor secuelae in this population. Calcium oral adminisrrarion was necesary for SIRI rime irs 2 pacients. Conclusions: Age at surgery was inadecuared in 4 parienta, probably due ro delayed admission. Basi calcirorsin levels were flor usefuhl. Due ro rhe anaromopathology findings, we enfasize rhe importan of famihial antecedents and molecular diagnosris ro perform toral prophylacric rhyroidecromy ar si early srage.