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Patient handout

Multiple endocrine neoplasia type 2A (MEN2A)

PRODUCTION

1. Your condition

This handout is for multiple endocrine neoplasia type 2a (men2a). Your care team identified this based on: positive germline ret mutation (proband or cascade-tested relative) (ata 2015).

Other reasons your team may use this plan: family history of men2 / mtc / pheochromocytoma (ata 2015); elevated basal/stimulated calcitonin or cea (ata 2015); medullary thyroid carcinoma or c-cell hyperplasia on fna / thyroid nodule (ata 2015).

2. Your medications

Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.

MedicationStarting doseHowWhenWhat it does
phenoxybenzamine10 mg BID, titrate q2–3 d to BP <130/80 + mild postural dropPOBID for 10–14 d preopATA 2015 / Endocrine Society 2014 — irreversible α-blockade must precede β; pheo excluded & treated before any MTC/parathyroid surgery
doxazosin1 mg daily → titrate to 8–16 mg/dayPOdailySelective α1 reversible alternative; preferred in pregnancy (ATA 2015)
propranolol20 mg TIDPOTIDβ ONLY after adequate α-blockade — never before (unopposed α → crisis) (ATA 2015)
phentolamine5 mg IV bolus, repeat q5–10 minIVPRNRapid IV α-blockade for intra/perioperative catecholamine crisis (ATA 2015)

Plan: MEN2A sequenced management — pheo FIRST (α→β) → thyroidectomy by ATA risk + calcitonin → parathyroidectomy → advanced-MTC RET inhibitor → surveillance (ATA 2015)

3. When to call your provider

Contact your care team if any of the following happen:

  • Pheo crisis / hypertensive emergency → ED/inpatient (ATA 2015)
  • Hypercalcaemic crisis → inpatient (ATA 2015)
  • Rapidly rising calcitonin / metastatic MTC → oncology + inpatient staging (ATA 2015)

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

  • Thyroid/parathyroid surgery, delivery, or anaesthesia planned in a MEN2A patient with unexcluded or unblocked pheochromocytoma (ATA 2015)(life-threatening)
  • BP ≥180/120 with end-organ damage or catecholamine cardiomyopathy in a MEN2A carrier (ATA 2015)(life-threatening)
  • Short calcitonin/CEA doubling time (<6–24 mo) or radiographically metastatic MTC (ATA 2015)
  • Corrected calcium >14 mg/dL or symptomatic hypercalcaemia (AMS, AKI, arrhythmia) from MEN2A primary hyperparathyroidism (ATA 2015)(life-threatening)
  • Pregnant MEN2A carrier with confirmed/suspected pheochromocytoma (ATA 2015)(life-threatening)

5. Follow-up

Genetic counselling + cascade RET testing of first-degree relatives; lifelong endo/surgery/genetics follow-up; pre-conception counselling (pheo exclusion before pregnancy); patient education on component surveillance and return precautions (ATA 2015)

6. Sources

Guideline: 2015 revised ATA Medullary Thyroid Carcinoma Guidelines (Wells); 2021-2025 MEN2/RET updates (selpercatinib LIBRETTO-001, pralsetinib ARROW; NCCN Neuroendocrine 2025)

  1. pubmed.ncbi.nlm.nih.gov/25810047
  2. pubmed.ncbi.nlm.nih.gov/32846061
  3. pubmed.ncbi.nlm.nih.gov/34118198