Adrenal lesions in a large kindred with multiple endocrine neoplasia type 1.
OBJECTIVE
To review the prevalence and natural history of adrenal lesions occurring in patients from a single kindred with multiple endocrine neoplasia type 1 (MEN-1).


METHODS
Case series.


METHODS
Tertiary referral center.


METHODS
Medical records of 33 patients from the Tasman 1 MEN-1 kindred who had undergone abdominal computed tomographic (CT) scanning were reviewed. In 30 patients, the results of abdominal ultrasonographic examinations were available for correlation with CT scan. Computed tomographic and ultrasound scans of 18 patients were reviewed by a radiologist blinded to the patients' clinical details. Three patients underwent adrenalectomy, and the histopathologic material was reviewed.


METHODS
Computed tomographic and ultrasound scans.


RESULTS
Adrenal lesions were detected in 12 patients (36%) by CT scan examination. Ultrasound imaged 58% of these lesions. Pancreatic lesions were present in all cases of adrenal disease. Follow-up was available for 8 patients with adrenal disease. Over 5.5 years, 6 patients (75%) had stable disease, 1 patient had an adrenal lesion that enlarged by 5 mm, and 1 patient had a lesion that enlarged by 50 mm. Adrenal histopathologic material was available in 3 patients. Macronodular cortical hyperplasia was present in 2 patients and a cortical adenoma present in 1 patient. Another kindred had bilateral macronodular cortical hyperplasia at autopsy.


CONCLUSIONS
Adrenal lesions are common in MEN-1 and occur in association with pancreatic disease. Abdominal CT scan is more sensitive than ultrasonographic examination in detecting adrenal disease. Primary hypersecretory syndromes of the adrenal glands appear to be rare, and the majority of lesions follow an indolent clinical course.
Burgess
J R
JR
Department of Diabetes and Endocrine Services, Royal Hobart Hospital, Australia.
Harle
R A
RA
Tucker
P
P
Parameswaran
V
V
Davies
P
P
Greenaway
T M
TM
Shepherd
J J
JJ
D000310:Adrenal Gland Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D000328:Adult; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000235:genetics* / Q000473:pathology; D010190:Pancreatic Neoplasms / Q000175:diagnosis / Q000235:genetics; D012680:Sensitivity and Specificity; D014057:Tomography, X-Ray Computed