Abstract
Medullary thyroid carcinoma (MTC) represents a distinct form of thyroid cancer with a potentially aggressive behavior, early lymph node metastases, and sporadic as well as hereditary forms. Only surgery with total thyroidectomy and lymph node dissection is able to achieve biochemical cure. Due to the uncommon nature of MTC, a specialization in endocrine surgery for diagnosis as well as therapy of MTC seem to be mandatory for a successful treatment. Knowledge of genotype-phenotype correlations for hereditary forms or routine calcitonin screening for sporadic forms is important to analyze preoperative findings and plan surgical procedures. Postoperative follow-up depends on biochemical cure or monitoring for patients with elevated calcitonin levels.