
Adrenal Lesions in a Large Kindred With Multiple Endocrine Neoplasia Type 1
John R. Burgess, MBBS;
Robin A. Harle, MBBS;
Paul Tucker, MD, FRCPA;
Venkateswaran Parameswaran, PhD;
Peter Davies, FRACR;
Tim M. Greenaway, PhD, FRACP;
Joseph J. Shepherd, MD
Arch Surg. 1996;131(7):699-702.
Abstract
 |  |
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).
Design Case series.
Setting Tertiary referral center.
Patients 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 scans. 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.
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. 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 I 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.
Arch Surg. 1996;131:699-702
Author Affiliations
From the Departments of Diabetes and Endocrine Services (Dr Burgess), Medical Imaging (Drs Harle and Davies), Anatomical Pathology (Dr Tucker), and Diabetes and Endocrine Services (Drs Parameswaran and Greenaway), Royal Hobart Hospital; and Department of Surgery, University of Tasmania (Dr Shepherd), Hobart, Australia.
THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES
 |
The appearance of the adrenal glands on computed tomography in multiple endocrine neoplasia type 1
Whitley et al.
Eur J Endocrinol 2008;159:819-824.
ABSTRACT
| FULL TEXT
Natural course of small adrenal lesions in multiple endocrine neoplasia type 1: an endoscopic ultrasound imaging study
Schaefer et al.
Eur J Endocrinol 2008;158:699-704.
ABSTRACT
| FULL TEXT
Molecular Markers and the Pathogenesis of Adrenocortical Cancer
Soon et al.
The Oncologist 2008;13:548-561.
ABSTRACT
| FULL TEXT
Multiple endocrine neoplasia type 1 in Northern Finland; clinical features and genotype phenotype correlation
Vierimaa et al.
Eur J Endocrinol 2007;157:285-294.
ABSTRACT
| FULL TEXT
Should Patients with Apparently Sporadic Pheochromocytomas or Paragangliomas be Screened for Hereditary Syndromes?
Jimenez et al.
J. Clin. Endocrinol. Metab. 2006;91:2851-2858.
ABSTRACT
| FULL TEXT
Hereditary Leiomyomatosis Associated with Bilateral, Massive, Macronodular Adrenocortical Disease and Atypical Cushing Syndrome: A Clinical and Molecular Genetic Investigation
Matyakhina et al.
J. Clin. Endocrinol. Metab. 2005;90:3773-3779.
ABSTRACT
| FULL TEXT
CONSENSUS: Guidelines for Diagnosis and Therapy of MEN Type 1 and Type 2
Brandi et al.
J. Clin. Endocrinol. Metab. 2001;86:5658-5671.
ABSTRACT
| FULL TEXT
Molecular and Genetic Mechanisms of Tumorigenesis in Multiple Endocrine Neoplasia Type-1
Guo and Sawicki
Mol. Endocrinol. 2001;15:1653-1664.
ABSTRACT
| FULL TEXT
Bilateral Pituitary Adenomas Occurring with Multiple Endocrine Neoplasia Type One
Sahdev and Jäger
Am. J. Neuroradiol. 2000;21:1067-1069.
ABSTRACT
| FULL TEXT
Complete Sequencing and Messenger Ribonucleic Acid Expression Analysis of the MEN I Gene in Adrenal Cancer
Schulte et al.
J. Clin. Endocrinol. Metab. 2000;85:441-448.
ABSTRACT
| FULL TEXT
Genotyping of Adrenocortical Tumors: Very Frequent Deletions of the MEN1 Locus in 11q13 and of a 1-Centimorgan Region in 2p16
Kjellman et al.
J. Clin. Endocrinol. Metab. 1999;84:730-735.
ABSTRACT
| FULL TEXT
|