Familial medullary thyroid carcinoma, pheochromocytoma, and parathyroid adenoma (Sipple's syndrome) Study of a kindred

Fourteen patients from a kindred are presented, 10 of whom had Sipple's syndrome (pheochromocytoma and medullary thyroid carcinoma) and 6 of whom had coexistent parathyroid adenomas or hyperplasia. There was a high incidence of bilateral involvement in both pheochromocytoma and medullary thyroid carcinoma, 70% and 92%, respectively. Parathyroid adenomas or chief cell hyperplasia was associated with chemical or clinical evidence of hyperparathyroidism in two thirds of patients. There was no mortality associated with pheochromocytoma; however, 3 of 14 patients studied died with medullary carcinoma, a 5‐year survival of 80%. Abdominal exploration with mobilization of both adrenal glands and excision as indicated, and total thyroidectomy with regional lymphadenectomy is the recommended treatment for this syndrome. The pathogenesis, clinical features, and hereditary pattern of Sipple's syndrome are discussed. The importance of the awareness of the features of this syndrome in the proper management of patients is stressed, and the performance of routine screening tests is recommended.