Multiple endocrine neoplasia II A syndrome: relationship between age of the patient, levels of basal calcitonin and size of the thyroid tumor]

This paper presents our experience in four families having the multiple endocrine neoplasia (MEN) II-A syndrome, with a total of 19 affected patients. All had medullary thyroid carcinoma (MTC), 6 also had pheochromocytoma (PH) and 3 had hyperparathyroidism. The screening of the members of the families to measure basal and pentagastrin response calcitonin (CT) serum levels allowed an early diagnosis of medullary thyroid carcinoma, when lesions were only 1 mm in diameter. Measurement of vanillymandelic acid, catecholamines and metanephrines in 24-hour urine collections allowed the diagnosis of pheochromocytoma in patients, some of whom were asymptomatic. A clear relationship was found between the age of the patients, the basal serum calcitonin level and size of the MTC.