Long-term biochemical results after operative treatment of primary hyperparathyroidism associated with multiple endocrine neoplasia types I and IIa: is a more or less extended operation essential?

OBJECTIVE: To analyse our long term results in patients operated on for primary hyperparathyroidism associated with multiple endocrine neoplasia types I and IIa.

DESIGN: Retrospective (data collection) and prospective (follow-up) analysis.

SETTING: University hospital, Germany.

SUBJECTS: 39 patients with MEN type I-associated and 7 patients with MEN-type-IIa-associated primary hyperparathyroidism.

INTERVENTIONS: Subtotal parathyroidectomy (n = 25 with MEN I and 1 with MEN IIa), total parathyroidectomy and autotransplantation (one in each group) and removal of only enlarged glands (13 with MEN type I and 5 with MEN type IIa).

MAIN OUTCOME MEASUREMENTS: Recurrence rate of hyperparathyroidism and permanent hypocalcaemia postoperatively.

RESULTS: Subtotal parathyroidectomy in patients with MEN type I gave a significantly lower recurrence rate than removal of only enlarged glands (3/25 compared with 3/13, log rank, p = 0.04). Permanent hypocalcaemia developed in 3/25 compared with 3/13, respectively. 2/5 patients with MEN type IIa developed recurrences after removal of only enlarged glands and the rate was higher than expected.

CONCLUSIONS: A more extensive operation is essential for patients with MEN type I; the rate of permanent hypocalcaemia is not increased, but the recurrence rate is reduced. Patients with MEN type IIa should be treated by excision of enlarged glands alone, but this may be extended to subtotal parathyroidectomy in patients with severe symptoms.