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Abstract
Fifteen patients with the diagnosis of multiple endocrine adenomatosis, type II, syndrome
(MEA II) were reported from a single center to discuss the dilemmas of early detection
and treatment of the adrenal medullary, thyroid, and parathyroid gland diseases. Ten
patients came from three families. Three of the patients died, none in hypertensive
crisis. Bilateral adrenal medullary disease was present in six patients. Five patients
with proved pheochromocytoma had hypertension. All had diagnostic urinary catecholamine
values. Nine normotensive patients without proved pheochromocytoma, but in a high-risk
category for adrenal medullary disease, have multiple suspicious urinary catecholamines
suggestive of adrenal medullary hyperplasia. Bilateral adrenalectomy is recommended
for proved adrenal medullary disease in the MEA II syndrome. Medullary carcinoma of
the thyroid gland was found in 13 patients and is believed to be present in two others.
Five of the proved cases were occult, being discovered by elevation of pentagastrin-stimulated
serum calcitonin levels, justifying total thyroidectomy. Parathyroid hyperplasia was
found in three patients with preoperative hypercalcemia and in four others with preoperative
normocalcemia. Conservative treatment of parathyroid gland hyperplasia in the MEA
II syndrome is substantiated. Metachronous phenotypic expression of the syndrome components
was significant.
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Article info
Footnotes
☆Presented at the Thirty-fourth Annual Meeting of the Central Surgical Association, Buffalo, N. Y., March 3–5 1977.
Identification
Copyright
© 1977 Published by Elsevier Inc.