ea0021p219 | Endocrine tumours and neoplasia | SFEBES2009
Worth Gabriella
, Palazzo Fausto
, Tolley Neil
, Robinson Stephen
, Cox Jeremy
, Williams Graham
, Bassett Duncan
MEN2B is the most aggressive form of MEN2. Consequently, the new American Thyroid Association guidelines recommend prophylactic thyroidectomy early in the first year of life. Ninety-seven percentage of MEN2B cases result from a germline methionine to threonine mutation at codon 918 (M918T) of the RET proto-oncogene. In addition, an exceedingly rare alanine to phenylalanine mutation at codon 883 (A883F) has been reported in 4 unrelated adults. In each case metastatic MTC and th...