Advances in the management of hereditary medullary thyroid cancer.
This work draws on recent advances during the era of codon-oriented prophylactic surgery for hereditary medullary thyroid cancer (MTC). Milestones included identification of RET (REarranged during Transfection) as the susceptibility gene, introduction of prophylactic surgery on evidence of a RET germline mutation, revelation of genotype-phenotype correlations within the MEN 2 spectrum and demonstration of age-related progression of MTC. Novel surgical techniques, notably systemic microdissection and compartment-oriented surgery, have greatly enhanced surgical cure. Uncovering molecular pathways from RET genotype to MEN 2 phenotype should provide treatment options for RET mutation carriers whose MTC currently is too advanced for cure.
Machens
A
A
Department of General, Visceral and Vascular Surgery, Martin-Luther-University Halle-Wittenberg, Halle/Saale, Germany.
Ukkat
J
J
Brauckhoff
M
M
Gimm
O
O
Dralle
H
H
D000367:Age Factors; D002116:Calcitonin / Q000097:blood; D003106:Colon / Q000601:surgery; D018450:Disease Progression; D005838:Genotype; D006801:Humans; D009377:Multiple Endocrine Neoplasia / Q000235:genetics / Q000473:pathology / Q000601:surgery; D009362:Neoplasm Metastasis / Q000473:pathology; D010641:Phenotype; D011518:Proto-Oncogene Proteins / Q000235:genetics; D051096:Proto-Oncogene Proteins c-ret; D020794:Receptor Protein-Tyrosine Kinases / Q000235:genetics; D013964:Thyroid Neoplasms / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D013965:Thyroidectomy / Q000379:methods
D011518:Proto-Oncogene Proteins; D002116:Calcitonin; D051096:Proto-Oncogene Proteins c-ret; D020794:Receptor Protein-Tyrosine Kinases