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

MEN1-Related Primary Hyperparathyroidism When Subtotal Parathyroidectomy Did Not Restore Normocalcaemia

In Multiple endocrine neoplasia type 1, persistent or recurrent primary hyperparathyroidism after the index parathyroid operation defines a specific clinical situation requiring a structured next step.

Clinical Scenario — Primary Hyperparathyroidism in MEN1
This protocol addresses MEN1-related primary hyperparathyroidism that is symptomatic, has evidence of target organ involvement, or where total serum calcium in adults is consistently more than 1 mg/dL (0.25 mmol/L) above the upper limit of the reference range.
Prior Line — When the Index Operation Has Not Met Its Goal
The initial approach — subtotal (3–3.5 gland) parathyroidectomy with concomitant transcervical thymectomy, performed by a high-volume MEN1 parathyroid surgeon — aims to restore normocalcaemia while avoiding permanent post-operative hypoparathyroidism. When that goal is not achieved, or when hypercalcaemia recurs, the protocol below defines the next step.
Next Step — Partial View Only
Management at this stage involves a surgical reintervention. Multimodal non-invasive imaging is used beforehand to localise the hyperfunctioning tissue. The complete protocol — including the specific reoperative strategy and the criteria for achieving near-normal serum calcium — is accessible via the link below.

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.
  • The indications for reintervention for persistent or recurrent MEN1-related primary hyperparathyroidism are generally the same as for index surgery, although there are important caveats.
  • Therefore, the procedure should be performed by an experienced parathyroid surgeon with expertise in managing MEN1-related primary hyperparathyroidism.
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