Management of hyperparathyroidism in the multiple endocrine neoplasia syndromes and other familial endocrinopathies.

Generalized parathyroid hyperplasia with superimposed clonal tumor growth is the most frequent expression of the MEN 1 trait and must be corrected surgically. The Zollinger-Ellison syndrome is an indication for early and aggressive control of hypercalcemia. If gastrin secretion is normal, early parathyroid surgery is usually not required; and to delay the operation will facilitate location of all parathyroid glands, a requisite for successful surgery. The best surgical approach is an extensive primary dissection including thymectomy, followed by total parathyroidectomy with autogenous parathyroid grafting. Parathyroid disease in MEN 2 is less frequent and of later onset. It usually takes secondary consideration to the cure of the C-cell neoplasm. Several other less common hereditary syndromes with parathyroid involvement are reviewed.