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Background. Medullary thyroid carcinoma (MTC)is a rare development of thyroid cancer with a nonegligible mortality rate. Our aim was to determinefactors that predict outcome in patients with MTC.Methods. We reviewed the records of all patientswith MTC (n = 56) who underwent treatment at ourinstitution between January 1990 and December2000. Univariate and multivariate analysis of clinicopathologicpredictors of MTC outcome were performedto identify subsets of patients with differentprobabilities in terms of overall survival, local recurrence,and distant metastases.Results. Multivariate analysis demonstrated that astatistically significant decrease in overall survivalis associated with T4b tumours (p = 0.06), the presenceof distant metastases at the time of presentation(p = 0.033), lymphatic invasion (p = 0.099), andpostoperative treatment (p = 0.045).Conclusions. The analysis of survival curves of patientswith MTC shows that the occurrence of locoregionaland distant metastases occurs preferentiallywithin the first 5 years, which identifies this asa crucial period for follow-up. In this series of patientswith MTC, the tumours classified as T4b,metastases at presentation, the presence of lymphovascularinvasion, and postoperative treatment werethe most important prognostic features. At present,there is no available beneficial adjuvant therapy.However, as the development of molecular therapyprogresses, it should be tested in clinical trials withthe purpose of achievement of novel targeted therapiesfor selected MTC patients with risk factors