What are the NCCN guidelines on the primary treatment of medullary thyroid carcinoma (MTC)?

Updated: Jun 24, 2020
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National Comprehensive Cancer Network (NCCN) guidelines recommend total thyroidectomy and bilateral central neck dissection (level VI) for all patients with medullary thyroid carcinoma (MTC) whose tumor is ≥1 cm or who have bilateral thyroid disease, as well as the following [5] :

  • Therapeutic ipsilateral or bilateral modified neck dissection for clinically or radiologically identifiable disease (levels II–V)
  • Prophylactic ipsilateral modified neck dissection for high volume or gross disease in the adjacent central neck may be considered

External beam radiation therapy (EBRT) is an option for treatment of incomplete tumor resection when further surgical resection is no longer possible. EBRT can also be considered for adjuvant treatment for extrathyroidal extension (T4a or T4b) with positive margins

Other therapy considerations are as follows:

  • Total thyroidectomy is recommended and neck dissection can be considered for those whose tumor is < 1 cm and for unilateral thyroid disease
  • Radioiodine ( 131I) therapy is not effective.
  • Suppression of thyroid-stimulating hormone (TSH) is not appropriate; TSH is kept in the normal range by adjusting levothyroxine dose.
  • Prior to thyroid surgery, pheochromocytoma removal by laparoscopic adrenalectomy is recommended, along with preoperative treatment with alpha-adrenergic blockade (phenoxybenzamine) or alpha-methyltyrosine to avoid a hypertensive crisis during surgery.

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