Medicine · Diabetes Mellitus and Endocrine Disorders (Thyroid, Adrenal, Pituitary, Parathyroid)

In a patient with MEN type 2A (RET proto-oncogene mutation at codon 634), which is the CORRECT sequence of prophylactic management?

  • A Total thyroidectomy first, then screen for pheochromocytoma
  • B Prophylactic parathyroidectomy before thyroidectomy to prevent postoperative hypocalcaemia
  • C Annual pentagastrin stimulation testing; operate only when calcitonin rises above 100 pg/mL
  • D Screen for pheochromocytoma first, then perform thyroidectomy
Correct answer: D. Screen for pheochromocytoma first, then perform thyroidectomy

Explanation

In MEN2A, pheochromocytoma must be excluded (or resected first) before thyroidectomy because general anaesthesia-induced hypertensive crisis can be fatal if a phaeochromocytoma is unsuspected. After ruling out or removing phaeochromocytoma, prophylactic total thyroidectomy is performed (timing depends on codon risk category). Parathyroid exploration follows only if hypercalcaemia is confirmed.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

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