Open AccessCurrent surgical management in RET mutation carriers [Aktualne postępowanie chirurgiczne u nosicieli mutacji proto-onkogenu RET]
Author(s) -
Agnieszka Czarniecka,
Małgorzata Oczko-Wojciechowska,
Anna Hajduk,
Marcin Zeman,
Barbara Jarząb
Publication year - 2019
Publication title -
endokrynologia polska
Language(s) - Uncategorized
Resource type - Journals
SCImago Journal Rank - 0.413
H-Index - 27
eISSN - 2299-8306
pISSN - 0423-104X
DOI - 10.5603/ep.a2019.0021
Subject(s) - medicine , calcitonin , medullary cavity , thyroid carcinoma , thyroidectomy , multiple endocrine neoplasia type 2 , medullary thyroid cancer , histopathological examination , medullary carcinoma , mutation , genetic testing , thyroid , oncology , pathology , germline mutation , gene , genetics , biology
Medullary thyroid carcinoma (MTC) still remains a rare endocrine tumor. 20-25% of MTC cases are genetically determined. The detection of the RET proto-oncogene mutation in 1993 allowed to understand the unique genotype-phenotype relationships in hereditary medullary thyroid carcinoma (HMTC) and formed the basis for therapeutic decisions based on the molecular results. Currently, prophylactic thyroidectomy is a commonly adopted and accepted therapeutic method. The decision on the time and extent of surgery should be made based on the results of molecular examination, the assessment of calcitonin (Ct) concentration and family history. Treatment of patients with HMTC requires the cooperation of a multidisciplinary team of experts and should be done in specialized centers only. The study is a review of the current guidelines for surgical management in the MEN2 syndrome.