0521779407-14 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:16
Multiple Endocrine Neoplasia 2 1015■Adrenalectomy prior to thyroidectomy if pheochromocytoma
present; alpha-blockade prior to beta-blockade to mitigate unop-
posed alpha vasoconstriction
follow-up
■Regularly assess for recurrent disease, esp MTC, because of high
metastatic potential
■Calcitonin (stimulated) annually for first 5 y after thyroidectomy,
then periodically
■24-h urine catecholamines and metanephrines 1 wk after adrenalec-
tomy
■Calcium after parathroidectomy
■24-hr urine catecholamines and metanephrines yearly
■Calcium yearly
complications and prognosis
Complications
■MTC:
➣Incidence of metastatic disease decreased with improved screen-
ing
➣Up to 95% cure rate with thyrroidectomy, many have local spread
➣Hypertensive crisis possible if not screened for pheochromocy-
toma
■Pheochromocytoma:
➣Surgical risk: 2–3% mortality, requires adequate adrenergic
blockade and fluid resuscitation with expert anesthetic care
➣Laparoscopic procedure reduces risk
➣Risk of contralateral pheochromocytoma 50% at 10 y
➣Adrenal insufficiency from bilateral adrenalectomy
■Parathyroidectomy:
➣Recurrent hyperparathyroidism common
➣Subsequent surgery guided with imaging and venous sampling
for PTHPrognosis
■Greatly improved with availability of genetic screening
■5-y survival for TNM I or II MTC 95%; delayed diagnosis dramatically
reduces survival
■Prognosis good after unilateral adrenalectomy; hypertension cured
in 75%
■Recurrent hyperparathyroidism less common than in MEN1