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KMID : 0371320000590010115
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Journal of the Korean Surgical Society
2000 Volume.59 No. 1 p.115 ~ p.123
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Multiple Endocrine Neoplasia Type IIa -Report of three cases-
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박제훈/Je Hoon Park
이해경/남석진/정재훈/이명식/양정현/Hae Kyung Lee/Seok Jin Nam/Jae Hoon Jung/Myung Shick Lee/Jung-Hyun Yang
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Abstract
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Purpose: Multiple endocrine neoplasia (MEN) type IIa is a syndrome composed of pheochromocytoma, a medullary thyroid carcinoma, and parathyroid hyperplasia with autosomal dominant inheritance. The syndrome is rare, but has clinical significance
due
to
high morbidity and mortality without early treatment. Early detection and treatment of the syndrome for high-risk familial members may improve life expectancy. Methods & Results: We experienced 3 cases of MEN type IIa. The first was in a 55-
year-old
woman who underwent total thyroidectomy and bilateral adrenalectomy. The second was in a 27-year-old man who was diagnosed with the disease based on CT findings of the abdomen. A bilateral subtotal adrenalectomy, total thyroidectomy, and subtotal
parathyroidectomy were performed. The third was in a 19-year-old woman with a familial history of MEN IIa who had a positive result on a c-ret protooncogene screening test. Recently, after documentation of the relationship between MEN IIa and
c-ret
protooncogene in genetic pathophysiology, the c-ret protooncogene screening test has been emphasized for high-risk familial members. Conclusion: We suggest that the use of c-ret protooncogene screening in high-risk familial members is important
in
the
early detection and treatment of MEN IIa, and consequently reduces morbidity and mortality.
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KEYWORD
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