Long-term follow-up of a kindred with multiple endocrine neoplasia type IIA

A kindred with multiple endocrine neoplasia type IIA was studied. In four of five fully documented cases, patients presented with adrenal medullary disease during the second or third decade; in three cases, medullary thyroid carcinoma was not diagnosed until the fourth decade. This kindred seems to have an aggressive variant of adrenal medullary disease in which clinical presentation is early, metastasis occurs, and oncological cure is uncommon. Early detection and operation were important, and symptoms were controlled with operations and alpha- and beta-adrenergic blockade in uncured members.