Eleven cases of pheochromocytoma, four of medullary thyroid carcinoma, two of parathyroid adenoma, and one of Cushing's syndrome have been demonstrated in a family that we have traced through seven generations.
The pheochromocytomas have been of adrenal origin, and all have been bilateral. In the majority of cases, there has been paroxysmal hypertension. However, two affected patients had recurrent, severe paroxysms of tachycardia, tremor, and headache during which the blood pressure was normal, illustrating one of the difficulties in the diagnosis of paroxysmal pheochromocytomas.
Provocative testing was performed in several patients. Intravenous histamine and glucagon proved to be about equally