Multiple endocrine neoplasia type 1
ICD-10 E31.8 · ICD-11 2F7A.0.1

Primary Hyperparathyroidism in Multiple Endocrine Neoplasia Type 1 — When Treatment Is Indicated

Clinical scenario

This protocol applies to patients with MEN1-related primary hyperparathyroidism who meet at least one of the following indications for intervention:

  • Symptomatic disease
  • Evidence of target organ involvement
  • Adults with total serum calcium consistently >1 mg/dL (0.25 mmol/L) above the upper limit of the reference range
Treatment approach
The indicated management is surgical, directed at the parathyroid glands, and must be performed by an experienced high-volume MEN1 parathyroid surgeon. The specific operative strategy — including the extent of gland removal and whether additional structures are addressed at the same time — varies by patient age and the anatomical distribution of disease. The complete operative algorithm is in the full protocol.
Clinical goals

Restore normocalcaemia and maintain near-normal serum calcium concentration for as long as possible, with as few operations as possible, while preventing target organ damage and avoiding permanent post-operative hypoparathyroidism.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1016/S2213-8587(25)00119-6
  • Parathyroidectomy is indicated in children, adolescents, and adults diagnosed with MEN1-related primary hyperparathyroidism who are symptomatic or have evidence of target organ involvement.
  • Irrespective of symptoms, parathyroidectomy is indicated in adults diagnosed with MEN1-related primary hyperparathyroidism, in whom total serum calcium levels are consistently >1 mg/dL (0·25 mmol/L) above the upper limit of the reference range of the specific assay used.
  • In children, adolescents, and adults with MEN1-related primary hyperparathyroidism, subtotal (3–3·5 gland) parathyroidectomy with concomitant transcervical thymectomy is the recommended index operation, which should be performed by an experienced MEN1 parathyroid surgeon.
  • In selected cases, unilateral clearance can be considered as the index operation for children or adolescents with MEN1-related primary hyperparathyroidism.
  • The goals of management are to maintain a (near) normal serum calcium concentration for as long as possible with as few operations as possible and preventing or limiting target organ damage while avoiding permanent post-operative hypoparathyroidism.
View source ↗