ALDOSTERONE-SECRETING ADRENAL ADENOMA AS PART OF MULTIPLE ENDOCRINE NEOPLASIA TYPE-1 (MEN1) - LOSS OF HETEROZYGOSITY FOR POLYMORPHIC CHROMOSOME-11 DEOXYRIBONUCLEIC-ACID MARKERS, INCLUDING THE MEN1 LOCUS

被引:79
作者
BECKERS, A
ABS, R
WILLEMS, PJ
VANDERAUWERA, B
KOVACS, K
REZNIK, M
STEVENAERT, A
机构
[1] STATE UNIV LIEGE, DEPT PATHOL, B-4000 LIEGE, BELGIUM
[2] STATE UNIV LIEGE, DEPT NEUROSURG, B-4000 LIEGE, BELGIUM
[3] UNIV ANTWERP, DEPT ENDOCRINOL, ANTWERP, BELGIUM
[4] UNIV ANTWERP, DEPT MED GENET, ANTWERP, BELGIUM
[5] UNIV TORONTO, DEPT PATHOL, TORONTO M5S 1A1, ONTARIO, CANADA
关键词
D O I
10.1210/jc.75.2.564
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
A 63-yr-old female presented with the extremely rare occurrence of an aldosterone-secreting adrenocortical adenoma as part of the syndrome of multiple endocrine neoplasia type 1 (MEN1). Only two other MENI patients were reported in the literature with hyperaldosteronism. The patient's MEN1 syndrome consisted of the association of primary hyperparathyroidism due to parathyroid adenoma, a prolactinoma, and a toxic multinodular goiter. Elevated basal and meal-stimulated serum PP levels without demonstrable pancreatic tumor were also found. Genetic analysis of the aldosterone-secreting adenoma with DNA markers localized on chromosome 11 showed loss of heterozygosity in tumor DNA. Since the MEN1 syndrome is caused by loss of the tumor suppressor gene on chromosome 11 in the llq13 region, it is probable that the same mechanism is associated with the formation of the adrenocortical adenoma.
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页码:564 / 570
页数:7
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