Early, Prophylactic Thyroidectomy in Hereditary Medullary Thyroid Carcinoma

• DOI: 10.1097/coc.0b013e3182a78fec
• Publication Date: 2013-10-07T15:39:47Z
• Authors: Maria R. Pelizzo; Francesca Torresan; Isabella M. Boschin; Davide Nacamulli; Gianmaria Pennelli; Susi Barollo; Domenico Rubello; Caterina Mian
• Keywords: Pentagastrin; Medullary carcinoma; Prophylactic Surgery

Prophylactic thyroidectomy has been encouraged for children with REarranged during Transfection (RET) germline mutations to prevent the onset, persistence, or recurrence of medullary thyroid carcinoma (MTC). The American Thyroid Association (ATA) recently published guidelines on timing prophylactic thyroidectomy. Our aim here was seek information optimal surgery carriers RET gene no clinical evidence disease, bearing in mind ATA recommendations.From 1986 2012, total performed at our institute 31 mutations, 28 them found family screening post-RET era, and other 3 under 20 years age classified as "early cases" pre-RET era. following parameters were studied: surgery, MTC risk, basal calcitonin (bCT) pentagastrin-stimulated (sCT), outcomes, persistence disease.By family, most prevalent mutation codon 634 (30%) mutation. youngest patient 5 old. Overall, 68% cases; 52% sample had a normal bCT 25% an sCT unresponsive pentagastrin. only factor predicting risk final histology ATA-RET level C. On receiver oparating curves analysis, cutoff over 24 predicted (P=0.06) yield resected specimen. Interestingly, none patients nodal involvement (0/21 MTC). Yet, permanent nerve palsy, 1 hypocalcemia. postoperatively follow-up all but patients.It is noteworthy that cancer removed 100% (9/9 patients, families) codons 891 768 (2/2 each 2 families those mutations), followed by 67% 609 (4/6 family), 60% 618 (3/5 4 In cases levels B C, waiting increase and/or may not guarantee before develops (which would assure life free diseases less-invasive surgical procedure, without any need central lymph-node dissection).