Penetrance of phaeochromocytoma in RET G533C carriers differs between South America and Europe

In a recent issue of Endocrine-Related Cancer, Castinetti et al. reported a different natural history of phaeochromocytoma in multiple endocrine neoplasia type 2 (MEN 2) patients between Europe and South America (Castinetti et al. 2017). The authors retrospectively collected data about the phaeochromocytoma in MEN 2 patients carrying RET mutations on exons 10 and 11 in 14 countries from South America, Southern Europe, Western Europe and Central Europe. They showed that phaeochromocytoma penetrance is lower (53%) in South America compared to South Europe, Western Europe and Central Europe.