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Medullary thyroid carcinoma (MTC) is a rare neuroendocrine neoplasm that arises from parafollicular C cells of the thyroid gland. Unlike the typical papillary carcinoma, medullary carcinoma develops more rapidly and causes distant metastases resistant to chemotherapy. The treatment options for MTC are surgery or pharmacotherapy. In recent years, major advances have been made especially in the pharmacological treatment of MTC. This paper aims to review the methods of diagnosis and treatment of MTC, taking into consideration the most recent findings. PubMed, PubMed Central, and Google Scholar databases were searched using keywords related to medullary thyroid carcinoma, its molecular and imaging diagnosis, thyroidectomy, and systemic pharmacotherapy. English-language articles were searched and selected after analyzing abstracts. Types of articles included mainly original articles and meta-analyses. From the initial search, 39 articles were retrieved for final analysis. This mini-review describes diagnostic methods for MTC focusing on testing biomarker levels. The most important, calcitonin, correlates linearly with tumor growth. First-line therapy for MTC is based on total thyroidectomy along with cervical lymphadenectomy. In some cases, systemic therapy based mainly on tyrosine kinase (TK) inhibitors is necessary. Research is also being conducted into gene therapy and blockage of tumor mitochondrial metabolism. MTC is a rapidly growing neuroendocrine tumor requiring radical surgical treatment. In cases where it is not possible, treatments that block the process of tumor carcinogenesis at various stages are used. New substances are being developed constantly to allow more effective treatment.
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