{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T15:11:35Z","timestamp":1770822695885,"version":"3.50.1"},"reference-count":66,"publisher":"American Academy of Pediatrics (AAP)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2003,2,1]]},"abstract":"<jats:p>Objectives. The aim of this study was first to investigate whether early total thyroidectomy (ETT; 1\u20135 years of age) can prevent medullary thyroid carcinoma with persistent or recurrent disease (PRD) in pediatric patients with multiple endocrine neoplasia type 2A (MEN-2A) and second, to evaluate the strength of codon analysis in children with MEN-2A as prognostic parameter.<\/jats:p>\n               <jats:p>Methods. Case reports and review of the literature for pediatric patients with MEN-2A were conducted. Inclusion criteria were age (0\u201320 years) and histologic degree of C-cell disease (normal = N, C-cell hyperplasia = CCH, medullary thyroid carcinoma = MTC, metastatic MTC = MMTC). To evaluate therapeutic results of ETT (1\u20135 years) versus late total thyroidectomy (LTT; 6\u201320 years), age-dependent histologic stages of C-cell disease and postoperative occurrence of PRD were compared. Prognostic value of specific codons, age-dependent histologic distribution, and long-term outcome were analyzed.<\/jats:p>\n               <jats:p>Results. In a total of 260 cases, 42 (16%) underwent ETT, and 218 (84%) underwent LTT. Histologic analysis showed significant difference between ETT versus LTT (57% vs 76%) regarding malignant stage of C-cell disease (of combined rate of MTC and MMTC). Long-term outcome was documented in 74 patients (28%). During a median follow-up period of 2 years (range: 0\u201315 years), 21 of 65 of the LTT group versus 0 of 9 of the ETT group suffered PRD. Information about codon analysis was available in 150 patients (58%). Mutated codons were c634 (63%), c618 (19%), c620 (9%), and c804 (6%). Codon-related histologic analysis resulted in prognostic differences: 81% of patients with c634-mutation had MCT or MMTC in contrast to c804 (44%), c618 (34%), and c620 (7%). Fifteen of 17 MMTC and 7 of 9 PRD occurred in patients with c634-mutation.<\/jats:p>\n               <jats:p>Conclusions. 1) ETT until 5 years of age in MEN-2A gene carriers results in significant reduction of MTC and MMTC in favor of CCH and improved disease-free long-term outcome. 2) Codon analysis is an important prognostic factor. Timing of TT could be individualized based on codon-specific prognosis. Until more detailed knowledge is available, consequent genetic and biochemical screening is mandatory for appropriate individual timing of ETT before age of 5 years.<\/jats:p>","DOI":"10.1542\/peds.111.2.e132","type":"journal-article","created":{"date-parts":[[2004,8,14]],"date-time":"2004-08-14T00:43:47Z","timestamp":1092444227000},"page":"e132-e139","source":"Crossref","is-referenced-by-count":60,"title":["Review of Multiple Endocrine Neoplasia Type 2A in Children: Therapeutic Results of Early Thyroidectomy and Prognostic Value of Codon Analysis"],"prefix":"10.1542","volume":"111","author":[{"given":"Gabor","family":"Szinnai","sequence":"first","affiliation":[{"name":"Department of Pediatric Endocrinology, University Children\u2019s Hospital, Basel, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christian","family":"Meier","sequence":"additional","affiliation":[{"name":"Division of Endocrinology, Department of Internal Medicine, University Hospital Basel, Basel, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul","family":"Komminoth","sequence":"additional","affiliation":[{"name":"Institute of Pathology, Kantonsspital Baden, Baden, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Urs W.","family":"Zumsteg","sequence":"additional","affiliation":[{"name":"Department of Pediatric Endocrinology, University Children\u2019s Hospital, Basel, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"417","published-online":{"date-parts":[[2003,2,1]]},"reference":[{"key":"2021121010101490200_R1","doi-asserted-by":"crossref","unstructured":"Sipple JH. 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