MEN2: surgical precision in the era of precision medicine.

Abstract

Graphical abstract:

Abstract: Medullary thyroid cancer, phaeochromocytoma and primary hyperparathyroidism in patients with multiple endocrine neoplasia type 2 can all be cured by surgery on the condition that they are detected early before locoregional or distant spread of malignant disease occurs and long term metabolic and structural damage to cardiovascular, renal and skeletal systems takes place. Recent scientific discoveries and technological advances made surgical decision process more precise and facilitated personalised treatments. RET analysis enables us to see this syndrome not as a monolith but as a cluster of different phenotypic presentations, each sending patient on an individual journey, which can be anticipated but not determined. Biochemical monitoring provides regular updates on transformation of endocrine cells in target endocrine organs and together with imaging helps to decide on time and extent of surgery. Advances in surgical technology allow for safer and less invasive interventions resulting in fewer complications, less trauma and better functional outcomes. Calibrating magnitude of surgery able to cure but do minimal harm, timing and performing it well is the art of the surgical precision in MEN2 patients. Surgical outcomes have improved in the last 30 years and we need to continue on this road. Precision in surgery aiming at near perfect surgical performance is achievable and this review looks at surgical decision making process through the prism of genetics and biochemical testing combined with imaging, former setting a trajectory for the disease progression with a fair degree of probability and latter assessing functional and structural changes over time.