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Multiple endocrine neoplasia type 1 and adrenal lesions
L Barzon1, C Pasquali, C Grigoletto
1Department of Medical and Surgical Sciences, University of Padova, Padova, Italy.
Purpose:
We investigated the relationship of long-term pancreatic hormone hypersecretion with adrenal lesions in patients with multiple endocrine neoplasia type 1 and in those with sporadic pancreatic endocrine tumors.
Materials And Methods:
We assessed the prevalence of adrenal lesions in 20 patients with multiple endocrine neoplasia type 1 and in a control group of 12 with sporadic pancreatic endocrine tumors. We also performed genetic testing for germline mutations of MEN1, the multiple endocrine neoplasia type 1 gene.
Results:
Adrenal lesions were common in multiple endocrine neoplasia type 1, accounting for 35% of cases. All adrenal lesions were nonfunctioning and benign. The relative risk of adrenal tumors was higher in patients with multiple endocrine neoplasia type 1 than in controls (p <0.05). No apparent relationship was observed of hormonal pattern or genotype with adrenal disease.
Conclusions:
Hormone hypersecretion by pancreatic endocrine tumors is not the primary cause of the development of adrenal lesions and the role of the MEN1 gene in adrenal tumorigenesis remains unclear. Adrenal lesions follow a benign course in most multiple endocrine neoplasia type 1 cases but careful morphological and functional followup is advisable.