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KMID : 0371319920420040568
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Journal of the Korean Surgical Society
1992 Volume.42 No. 4 p.568 ~ p.574
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Partial Adrenalectomy for Bilateral Pheochromocytoma in Multiple Endocrine Neoplasia Type Iia with Maintenance of Adrenocortical Function
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Abstract
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Multiple endocrine neoplasia, type IIa(MEN type IIa) is an inherited syndrome characterized by the occurrence of medullary thyroid carcinoma, parathyroid hyperphasia and adrenal pheochromocytoma. The adrenal medullary disease varies from
hyperplasia(diffuse or nodural or both) to bilateral multiple pheochromocytomas. The pathologic finding of the adrenal gland are almost always bilateral. Standard surgical treatment for bilateral pheochromocytoma consists of total adrenalectomy.
As
a
result of such a procedure all patients were required eventually life-long glucocortical and mineralocortical therapy.
Recently several studies have reported about different surgical approaches to preseve normal adrenocortical function postoperatively. We have experienced the case with MEN type IIa in 33-year-old female patient. She has undergone right total
adrenalectomy and left partial ad enalectomy saving a small amount of the left adrenal cortex, followed by a total thyroidectomy. Postoperatively, she has maintained normal blood pressure, along with preservation of adrenocortical funtion. On the
follow
up studies, there were no evidence of recurrence and she has been doing well for 24 months.
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KEYWORD
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