J Korean Surg Soc.  2000 Jul;59(1):115-123.

Multiple Endocrine Neoplasia Type IIa Report of three cases

• Park JH
• Lee HK
• Nam SJ
• Jung JH
• Lee MS
• Yang JH

Abstract

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.

Keyword

Multiple endocrine neoplasia type IIa; Pheochromocytoma; Medullary carcinoma; Thyroid neoplasms; Proto-oncogenes; Proto-oncogene proteins

MeSH Terms

Abdomen
Adrenalectomy
Adult
Carcinoma, Medullary
Female
Humans
Hyperplasia
Life Expectancy
Male
Mass Screening
Mortality
Multiple Endocrine Neoplasia Type 2a
Multiple Endocrine Neoplasia*
Parathyroidectomy
Pheochromocytoma
Proto-Oncogene Proteins
Proto-Oncogenes
Thyroid Neoplasms
Thyroidectomy
Wills
Young Adult
Proto-Oncogene Proteins