A Nationwide study of multiple endocrine neoplasia type 2A in Norway. Predictive avd prognostic factors for the clinical course of medullary thyroid carcinoma.

A Nationwide study of multiple endocrine neoplasia type 2A in Norway. Predictive avd prognostic factors for the clinical course of medullary thyroid carcinoma.

Thyroid. 2016 Jul 11;

Authors: Opsahl EM, Schlichting E, Helset K, Svartberg J, Brauckhoff K, Mæhle L, Engebretsen LF, Sigstad E, Grøholt K, Akslen LA, Jørgensen LH, Varhaug JE, Bjøro T

Abstract
BACKGROUND: Multiple endocrine neoplasia type 2A (MEN 2A) is an autosomal dominant syndrome caused by activating germline mutations in the RET (REarranged during Transfection) proto-oncogene. MEN 2A has a strong (>95%) and age dependent (5-25 years) clinical penetrance of medullary thyroid carcinoma (MTC). Several major studies have analyzed the predictive and prognostic factors for MEN 2A to find indicators that predict the optimal timing of prophylactic thyroidectomy. The aim of this study was to describe all known RET positive MEN 2A patients diagnosed in Norway and evaluate the clinical course of MTC, as well as its predictive and prognostic factors.
METHODS: This nationwide retrospective cohort study included data for 65 (14 index and 51 screening patients) out of a total of 67 MEN 2A patients with the RET gene mutation who were diagnosed in Norway since 1974. Data were collected by reviewing patient files. The variables analyzed were genotype, phenotype, preoperative basal calcitonin, age at thyroid surgery, central lymph node dissection and nodal status at primary surgery, number of surgical procedures, and biochemical cure. Of the 65 patients, 60 had undergone thyroid surgery. The median follow-up period was 9.9 years. The patients were divided into pre-RET-and RET-era, which included patients who had thyroid surgery before January 1, 1994 and after, respectively.
RESULTS: In index and screening patients, MTC was found, respectively, in 100% vs. 45% of cases, central lymph node dissection at primary surgery was done for 64% vs. 52% of patients, and the median total number of surgical procedures was 2 (range 1-6) vs. 1 (range 1-4). At primary surgery, all patients (13) with lymph node metastases had preoperative basal calcitonin levels ≥ 68 pg/ml, and all patients (17) without central lymph node dissection and preoperative basal calcitonin < 40 pg/mL were biochemically cured. Multivariate analysis showed that preoperative basal calcitonin was a significant predictive factor for MTC superior to age at thyroid surgery when analyzing the entire period (p=0.009) and the RET-era separately (p=0.021). Prognostic factors for biochemical cure were preoperative basal calcitonin, central lymph node dissection and nodal status at primary surgery (p=0.037/p=0.002/p=0.005) when analyzing the entire….

PMID: 27400880 [PubMed - as supplied by publisher]

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