Multiple endocrine neoplasia type 1 and adrenal lesions.
OBJECTIVE
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.


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.
Barzon
L
L
Department of Medical and Surgical Sciences, University of Padova, Padova, Italy.
Pasquali
C
C
Grigoletto
C
C
Pedrazzoli
S
S
Boscaro
M
M
Fallo
F
F
D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000175:diagnosis / Q000453:epidemiology*; D000328:Adult; D017677:Age Distribution; D000368:Aged; D016022:Case-Control Studies; D016009:Chi-Square Distribution; D015331:Cohort Studies; D015897:Comorbidity; D016001:Confidence Intervals; D005260:Female; D006801:Humans; D007558:Italy / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000175:diagnosis / Q000453:epidemiology*; D015995:Prevalence; D011336:Probability; D011379:Prognosis; D012307:Risk Factors; D017678:Sex Distribution; D018709:Statistics, Nonparametric