In MEN2A syndrome, the order of priority in which the three component tumours should be surgically addressed to prevent life-threatening complications is:

• A Pheochromocytoma → Medullary thyroid carcinoma → Primary hyperparathyroidism ✓
• B Medullary thyroid carcinoma → Pheochromocytoma → Primary hyperparathyroidism
• C Primary hyperparathyroidism → Pheochromocytoma → Medullary thyroid carcinoma
• D Medullary thyroid carcinoma → Primary hyperparathyroidism → Pheochromocytoma

Explanation

In MEN2A, pheochromocytoma must be excluded and surgically addressed first, before any elective procedure, to avoid hypertensive crisis during thyroid or parathyroid surgery. After adrenalectomy, total thyroidectomy with central neck dissection is performed for medullary thyroid carcinoma. Primary hyperparathyroidism (present in ~20%) is addressed last, often at the time of thyroid surgery. Operating on other lesions before treating the phaeochromocytoma can be fatal.

Reference: Bailey & Love's Short Practice of Surgery, 27th ed.

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Written and medically reviewed by the StethoPrep medical team.