DILEMMAS IN EARLY DIAGNOSIS AND TREATMENT OF MULTIPLE ENDOCRINE ADENOMATOSIS, TYPE-2

  • 1 January 1977
    • journal article
    • research article
    • Vol. 82  (3) , 407-413
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 disease. Ten patients came from 3 families. Three of the patients died, none in hypertensive crisis. Bilateral adrenal medullary disease was present in 6 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, had 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 2 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 3 patients with preoperative hypercalcemia and in 4 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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