TY - JOUR
T1 - Prognostic factors of disease-free survival after thyroidectomy in 170 young patients with a RET germline mutation
T2 - A multicenter study of the Groupe Français d'Etude des Tumeurs Endocrines
AU - Rohmer, Vincent
AU - Vidal-Trecan, G.
AU - Bourdelot, A.
AU - Niccoli, P.
AU - Murat, A.
AU - Wemeau, J. L.
AU - Borson-Chazot, F.
AU - Schvartz, C.
AU - Tabarin, A.
AU - Chabre, O.
AU - Chabrier, G.
AU - Caron, P.
AU - Rodien, P.
AU - Schlumberger, M.
AU - Baudin, E.
PY - 2011/3/1
Y1 - 2011/3/1
N2 - Background: In hereditary medullary thyroid carcinoma (HMTC), prophylactic surgery is the only curative option, which should be properly defined both in time and extent. Objectives: To identify and characterize prognostic factors associated with disease-free survival (DFS) in children from HMTC families. Design: We conducted a retrospective analysis of a multi-center cohort of 170 patients below age 21 at surgery. Demographic, clinical, genetic, biological data [basal and pentagastrine-stimulated calcitonin (CT and CT/Pg, respectively)], and tumor node metastasis (TNM) status were collected. DFS was assessed based on basal CT levels. Kaplan-Meier curves, Cox regression, and logistic regression models were used to determine factors associated with DFS and TNM staging. Results: No patients with a preoperative basal CT <31 ng/ml had persistent or recurrent disease. Medullary thyroid carcinoma defined by a diameter ≥10 mm [hazard ratio (HR): 6.0; 95% confidence interval (95%CI): 1.8-19.8] and N1 status (HR: 20.8;95%CI: 3.9-109.8) were independently associated with DFS. Class D genotype [odds ratio (OR): 48.5, 95% CI: 10.6-225.1], preoperative basal CT>30 ng/liter (OR: 43.4, 95% CI: 5.2-359.8), and age >10 (OR: 5.5, 95% CI: 1.4-21.8) were associated with medullary thyroid carcinoma ≥10mm. No patient with a preoperative basal CT <31 ng/ml had a N1 status. Class D genotype (OR: 48.6, 95% CI: 8.6-274.1), and age >10 (OR: 4.6, 95% CI: 1.1-19.0) were associated with N1 status. Conclusion: In HMTC patients, DFS is best predicted by TNM staging and preoperative basal CT level below 30 pg/ml. Basal CT, class D genotype, and age constitute key determinants to decide preoperatively timely surgery.
AB - Background: In hereditary medullary thyroid carcinoma (HMTC), prophylactic surgery is the only curative option, which should be properly defined both in time and extent. Objectives: To identify and characterize prognostic factors associated with disease-free survival (DFS) in children from HMTC families. Design: We conducted a retrospective analysis of a multi-center cohort of 170 patients below age 21 at surgery. Demographic, clinical, genetic, biological data [basal and pentagastrine-stimulated calcitonin (CT and CT/Pg, respectively)], and tumor node metastasis (TNM) status were collected. DFS was assessed based on basal CT levels. Kaplan-Meier curves, Cox regression, and logistic regression models were used to determine factors associated with DFS and TNM staging. Results: No patients with a preoperative basal CT <31 ng/ml had persistent or recurrent disease. Medullary thyroid carcinoma defined by a diameter ≥10 mm [hazard ratio (HR): 6.0; 95% confidence interval (95%CI): 1.8-19.8] and N1 status (HR: 20.8;95%CI: 3.9-109.8) were independently associated with DFS. Class D genotype [odds ratio (OR): 48.5, 95% CI: 10.6-225.1], preoperative basal CT>30 ng/liter (OR: 43.4, 95% CI: 5.2-359.8), and age >10 (OR: 5.5, 95% CI: 1.4-21.8) were associated with medullary thyroid carcinoma ≥10mm. No patient with a preoperative basal CT <31 ng/ml had a N1 status. Class D genotype (OR: 48.6, 95% CI: 8.6-274.1), and age >10 (OR: 4.6, 95% CI: 1.1-19.0) were associated with N1 status. Conclusion: In HMTC patients, DFS is best predicted by TNM staging and preoperative basal CT level below 30 pg/ml. Basal CT, class D genotype, and age constitute key determinants to decide preoperatively timely surgery.
UR - http://www.scopus.com/inward/record.url?scp=79952292850&partnerID=8YFLogxK
U2 - 10.1210/jc.2010-1234
DO - 10.1210/jc.2010-1234
M3 - Article
C2 - 21190982
AN - SCOPUS:79952292850
SN - 0021-972X
VL - 96
SP - E509-E518
JO - Journal of Clinical Endocrinology and Metabolism
JF - Journal of Clinical Endocrinology and Metabolism
IS - 3
ER -