| Abstract |
Medullary thyroid carcinoma (MTC) is a neuroendocrine tumour which arises
from calcitonin-producing parafollicular C-cells and accounts for 5% to
10% of all thyroid cancers, with an estimated age-adjusted annual
incidence of 0.18 for men and 0.23 for women per 100.000 inhabitants. MTC
occurs as either a sporadic form or in a familial context (25% of cases).
Familial forms are inherited in an autosomal-dominant way, either as part
of multiple endocrine neoplasia syndrome type 2A (MEN 2A) or 2B (MEN 2B),
or without any associated endocrinopathies as familial MTC (FMTC).
Surgical removal of all neoplastic tissue in the neck by total
thyroidectomy, central and bilateral neck dissection is the only
potentially curative treatment in localized disease. However, at the time
of initial DIagnosis, at least one quarter of patients has distant
metastases. MTC research is a major focus of Institute for Pathophysiology
and Immunology during the last 2.5 decades: 9 MTC-derived cell lines have
been established and characterized. These approved cell lines are
constantly used to study the MTC biology and to test therapeutic agents.
The aim of this work is to give an overview about the MTC tumour marker
and the new therapeutic strategies and drugs for the treatment of MTC.
Furthermore, the aim is to assemble the data provided about MTC cell lines
and to give an overview of their characteristics.
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