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.

Nyckelord

age-related progression

calcitonin

monitoring

medullary thyroid cancer

MEN type 2

nodal metastasis

RET proto-oncogene.

Publikations- och innehållstyp

ref (ämneskategori)

art (ämneskategori)