Onkologie. 2011:5(6):336-339
Medullarythyroidcarcinoma (MTC) accountsfor 8–10 % of allthyroidcancers. MTC ismainlysporadic, but anhereditarypattern (multipleendocrineneoplasia
type 2 (MEN 2)) ispresent in 25–30 % of cases, as anautosomal-dominant traitdue to germlinemutations of the
RET proto-oncogene. Theprimarytreatment of bothhereditary and sporadicforms of MTC istotalthyroidectomy and removal of allneoplastictissuepresent.
Aftersurgery, measurements of serumcalcitonin as a tumourmarkerand carcinoembryonic antigen are markers
in thepostsurgicalfollow-up of patientswith MTC as theyreflectthe presence of persistentorrecurrentdisease. Completeremissionisdemonstrated
by undetectable and stimulatedserumcalcitoninmeasurement. In patiens withelevatedbasalcalcitoninorafterstimulation
are performedimagingmethods (ultrasound of theneck, CT, scintigraphy, PET/CT).Metastasesoutsidetheneckmayoccur in the liver,
lungs, bones and, lessfrequently, brain. Surgeryisthemaintreatmentforlocal and distantmetastaseswheneverfeasible. In generalisation
of dinase are performedtheraphywith131I-MIBG in somecases. Systemicchemotherapyhas very limited efficacyorduration. Familialcasesmustbeidentified
by searchingfor RET proto-oncogenemutations in the proband and in familymembers.
Published: December 1, 2011 Show citation