Lessons Learned from the Management of a Rare Genetic Cancer

Less than a decade after the identification of activating mutations of the rearranged during transfection (RET) proto-oncogene in multiple endocrine neoplasia type 2 (MEN-2), a clinical syndrome characterized by medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism, germ-line genetic testing has become the basis for therapeutic decisions in affected patients. Indeed, consensus was reached at the International Multiple Endocrine Neoplasia meeting in Gubbio, Italy, in 1999 that the decision to perform a thyroidectomy in a young child should be based on the presence or absence of a germ-line RET mutation.1 Such rapid progress to consensus is remarkable in any scientific . . .