[Familial thyroid carcinomas].
Approximately 5% of differentiated thyroid carcinomas with follicular cell differentiation, papillary thyroid carcinoma (PTC) and follicular thyroid carcinoma (FTC) and 25-30% of medullary thyroid carcinoma (MTC) are hereditary. They occur either as part of a defined syndrome or are confined to the thyroid gland. Compared to their sporadic non-hereditary counterparts hereditary thyroid carcinomas generally develop earlier and regularly show multifocal tumour growth. With the exception of familial MTC, which is preceded by neoplastic C cell hyperplasia, no precursor lesions of hereditary thyroid carcinoma are known. In strong correlation with the localisation of the germline mutation of the RET protooncogene, familial MTC shows a distinct clinical course which allows precise clinical decision-making for prophylactic thyroidectomy to prevent invasive MTC. According to current knowledge prophylactic thyroidectomy of all other types of hereditary thyroid carcinoma is not justified.
Schmid
K W
KW
Institut für Pathologie und Neuropathologie, Universitätsklinikum Essen, Universität Duisburg-Essen, Hufelandstr. 55, 45122, Essen. kw.schmid@uk-essen.de
Ting
S
S
Sheu
S-Y
SY
D018263:Adenocarcinoma, Follicular; D002277:Carcinoma; D002291:Carcinoma, Papillary; D056733:Carney Complex / Q000235:genetics / Q000473:pathology; D005736:Gardner Syndrome / Q000235:genetics / Q000473:pathology; D005799:Genes, Dominant; D005808:Genes, Recessive; D018095:Germ-Line Mutation; D006223:Hamartoma Syndrome, Multiple / Q000235:genetics / Q000473:pathology; D006801:Humans; D009361:Neoplasm Invasiveness / Q000517:prevention & control; D013577:Syndrome; D000077273:Thyroid Cancer, Papillary; D013964:Thyroid Neoplasms / Q000235:genetics* / Q000473:pathology* / Q000601:surgery; D013965:Thyroidectomy