ea0090p386 | Endocrine-related Cancer | ECE2023
Vrckovnik Rok
, Kocjan Tomaž
Background: Multiple endocrine neoplasia type 2A (MEN2A) is a rare genetic tumor syndrome due to germline mutations of the RET proto-oncogene, which is characterized by medullary thyroid cancer (MTC), pheochromocytoma (PHEO), and primary hyperparathyroidism (PHPT). The age at onset, aggressiveness of MTC, and the penetrance of other components depend on the genotype. Early prophylactic thyroidectomy and life-long screening for PHEO from childhood are recommended in most cases....