ADRENAL LESIONS IN A LARGE KINDRED WITH MULTIPLE ENDOCRINE NEOPLASIA TYPE-1

Objective: To review the prevalence and natural history of adrenal les ions occurring in patients from a single kindred with multiple endocri ne neoplasia type 1 (MEN-1). Design: Case series. Setting: Tertiary re ferral center. Patients: Medical records of 33 patients from the Tasma n 1 MEN-1 kindred who had undergone abdominal computed tomographic (CT ) scanning were reviewed. In 30 patients, the results of abdominal ult rasonographic examinations were available for correlation with CT scan s. Computed tomographic and ultrasound scans of 18 patients were revie wed by a radiologist blinded to the patients' clinical details. Three patients underwent adrenalectomy, and the histopathologic material was reviewed. Main Outcome Measures: Computed tomographic and ultrasound scans. Results: Adrenal lesions were detected in 12 patients (36%) by CT scan examination. Ultrasound imaged 58% of these lesions. Pancreati c lesions were present in all cases of adrenal disease. Follow-up was available for 8 patients with adrenal disease. Over 5.5 years, 6 patie nts (75%) had stable disease, 1 patient had an adrenal lesion that enl arged by 5 mm, and 1 patient had a lesion that enlarged by 50 mm. Adre nal 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 cort ical hyperplasia at autopsy. Conclusions: Adrenal lesions are common i n 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 glan ds appear to be rare, and the majority of lesions follow an indolent c linical course.