S.B. Pinskiy1, V.A. Beloborodov1, V.V. Dvornichenko2, Yu.K. Batoroev2
1Irkutsk State Medical University, Irkutsk
2Irkutsk State Medical Academy of Postgraduate Education ― Branch Campus of the FSBEI FPE RMACPE MOH Russia, Irkutsk
Pinskiy S.B. ― D. Sc. (Medicine), Professor of General Surgery and Anesthesiology Department of Irkutsk State Medical University
118 Baikalskaya Str., Irkutsk, Russian Federation, 664046, tel. (3952) 70-37-29
Abstract. Medullary carcinoma (MR) is the only tumor of the thyroid gland (CH), which develops from parafollicular C-cells, which are part of the diffuse neuroendocrine system, which mainly synthesize calcitonin, as well as polypeptides and biologically active substances. MR is rare and ranges from 2 to 10% of the number of patients with malignant thyroid tumors. From 2000 to 2018, 3030 patients were operated on for thyroid cancer in the Irkutsk Regional Oncology Center, 205 (6.8%) of them were diagnosed with MR. There are 2 clinical forms of PH; sporadic and hereditary. Sporadic forms make up about 70% of MR, do not have a genetic origin or a characteristic type of inheritance. Preoperative diagnosis of MR is difficult due to the lack of typical clinical manifestations and the lack of information content of traditional research methods. The most reliable and effective method for the primary diagnosis of MR is the determination of serum calcitonin concentration, which is a specific marker. The removal of the thyroid gland before the development of MR, performed in early childhood, based on a positive genetic study, has a better clinical outcome than after thyroidectomy for an already developed disease. Modern methods of radiotherapy and chemotherapy are not very effective for PHT. Survival of patients depends on the size of the tumor, the adequacy of the operation, the presence or absence of metastases. A comprehensive study allows us to identify hereditary variants, promptly diagnose familial and sporadic forms of MR, and promptly carry out radical interventions.
Key words: medullary thyroid cancer, hereditary, sporadic, RET gene germline mutation.