Clinical Features of Multiple Endocrine Neoplasia Type I in Koreans
- Author:
Chul Hee KIM
;
Ghi Su KIM
;
Hong Kyu KIM
;
Joong Yeol PARK
;
Young Kee SHONG
;
Ki Up LEE
- Publication Type:Original Article
- Keywords:
Multiple endocrine neoplasia type I(MEN I);
Koreans
- MeSH:
Acromegaly;
Adenoma;
Asian Continental Ancestry Group;
Calcium;
Carcinoid Tumor;
Chungcheongnam-do;
Diagnosis;
Follow-Up Studies;
Gastrins;
Glucagon;
Humans;
Hyperparathyroidism;
Hyperplasia;
Hypogonadism;
Insulin;
Insulinoma;
Islets of Langerhans;
Mass Screening;
Multiple Endocrine Neoplasia Type 1;
Multiple Endocrine Neoplasia;
Peptic Ulcer;
Pituitary Neoplasms;
Prognosis;
Prolactinoma;
Retrospective Studies;
Urinary Calculi
- From:Journal of Korean Society of Endocrinology
1996;11(2):163-174
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Background: Multiple endocrine neoplasia type I(MEN I) is a rare, eomplex, and potentially lethal disease. In Korean, only five anecdotal cases were reported as yet. The authors rescently experienced four cases of MEN I, and analysed the clinieal characteristics of MEN I in Koreans. Methods: The authors evaluated nine cases of MEN I, retrospectively. Four cases were analysed by clinical records in patients admitted to Asan Medical Center and five cases were reviewed by previously reported Korean literature from 1986 to 1995. Results: The average age was 39 years(ranged from 33 to 59 years). Eight of the nine patients had hyperparathyroidism documented by elevated serum calcium and PTH level with or without evidence of parathyroid mass. Initial presenting manifestations were symptomatic urinary stone, hypoglycernia due to insulinoma, hypogonadism, acromegaly, or peptic ulcer. Eight of nine patients had pancreatic islet cell tumors, and three of them were be malignant by radiologic and/or pathologic findings. The pancreatic tumors produced various hormones, such as gastrin, insulin, glucagon, or combination of them. Six of the nine cases had pituitary lesion. The most cornmon pituitary tumor was prolactinoma and the remaining was GH or GH and TSH producing tumor. In addition to the major components of MEN I, four had adrenocortical hyperplasia or adenoma and two had carcinoid tumor. There was only one familial case. Conclusion: The clinical charateristics of MEN I in Korean are mostly not different from the previous reports except older age at diagnosis, more comrnon adrenal involvement(44%) and gastrointestinal carcinoid tumor(22%). Although only one case was familial, more cases could be found if careful screening were done for the family members of the MBN I patients. In addition, screening and close follow up for endocrine pancreatic tumors are required for MEN I patients without detectable pancreatic lesion becau~se the malignant potential of pancreatic tumors has beeome an increasing concem for the prognosis of MEN I.
