Patient handout

Multiple endocrine neoplasia type 1 (MEN1)

PRODUCTION

1. Your condition

This handout is for multiple endocrine neoplasia type 1 (men1). Your care team identified this based on: first-degree relative with men1 / known germline men1 mutation — cascade screening (thakker jcem 2012).

Other reasons your team may use this plan: primary hyperparathyroidism, especially age <40 or multiglandular disease (thakker jcem 2012); two or more of parathyroid / pancreatic-duodenal net / pituitary tumors (thakker jcem 2012 diagnostic criteria); recurrent / refractory peptic ulcer or secretory diarrhea — zollinger-ellison from gastrinoma (thakker jcem 2012).

2. Your medications

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

Medication	Starting dose	How	When	What it does
subtotal or total parathyroidectomy with cryopreservation	—	surgical	—	Thakker JCEM 2012 — MEN1 pHPT is multiglandular; less-than-subtotal resection has high recurrence. Subtotal (3.5-gland) or total parathyroidectomy with transcervical thymectomy + parathyroid tissue cryopreservation; balance recurrence vs permanent hypoparathyroidism
cinacalcet	30 mg	PO	daily, titrate to BID	Thakker JCEM 2012 — lowers calcium when surgery deferred/declined or for persistent disease; does not treat the underlying multiglandular hyperplasia
cholecalciferol	1000-2000 IU	PO	daily	Thakker JCEM 2012 — cautious repletion of concomitant vitamin D deficiency optimizes interpretation and bone health

Plan: MEN1 organ-directed ladder (parathyroid → gastrinoma → insulinoma → pituitary → carcinoid surveillance/resection)

3. When to call your provider

Contact your care team if any of the following happen:

Hypercalcemic crisis (Ca >14 / AKI / AMS) -> ED/inpatient (Thakker JCEM 2012)
Neuroglycopenic insulinoma episode -> inpatient localization + surgery (Thakker JCEM 2012)
Complicated Zollinger-Ellison (bleed/perforation) -> inpatient + IV PPI (Thakker JCEM 2012)
Pituitary apoplexy / chiasmal compression -> urgent neurosurgery (Thakker JCEM 2012)

4. When to seek emergency care

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

MEN1 pHPT with calcium >14 mg/dL and AKI / altered mental status / arrhythmia(life-threatening)
Endogenous hyperinsulinemic hypoglycemia with neuroglycopenic symptoms (confusion, seizure, loss of consciousness)(life-threatening)
Gastrinoma with refractory ulcer disease, GI hemorrhage, or perforation
Thymic carcinoid detected on chest imaging (invasive/large), especially male smoker
Pituitary macroadenoma with apoplexy, acute visual loss, or chiasmal compression(life-threatening)
Functional pancreatic NET with metastatic disease or rapid growth (>2 cm / G2-G3)

5. Follow-up

Cascade genetic testing + counseling for at-risk first-degree relatives (offer from ~5-10 y of age depending on earliest familial tumor), patient education, smoking cessation (thymic risk), pregnancy planning, and lifelong MEN1-center follow-up (Thakker JCEM 2012)

6. Sources

Guideline: 2012 Endocrine Society MEN1 Clinical Practice Guideline (Thakker); 2021-2025 MEN1 surveillance updates