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Adrenal Lesions in a Large Kindred With Multiple Endocrine Neoplasia Type 1

Adrenal Lesions in a Large Kindred With Multiple Endocrine Neoplasia Type 1 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 References 1. Wermer P. Genetic aspects of adenomatosis of endocrine glands . Am J Med . 1954;16:363-371.Crossref 2. Bystrom C, Larsson C, Blomberg C, et al. Localisation of the MEN 1 gene to a small region within chromosome 11q13 deletion mapping in tumours . Proc Natl Acad Sci USA . 1990;87:1968-1990.Crossref 3. Knudson AG. Mutation and cancer: statistical study of retinoblastoma . Nature . 1971;332:85-87. 4. Brandi ML, Marx SJ, Aurbach GD, Fitzpatrick LD. Familial multiple endocrine neoplasia type 1: a new look at pathophysiology . Endocr Rev . 1987;8:391-405.Crossref 5. Gross MD, Shapiro B. Clinically silent adrenal masses . J Clin Endocrinol Metab . 1993;77:885-888. 6. Skogseid B, Larsson C, Lindgren P, et al. Clinical and genetic features of adrenocortical lesions in multiple endocrine neoplasia type 1 . J Clin Endocrinol Metab . 1992;75:76-81. 7. Ballard HS, Frame B, Hartsock RJ. Familial multiple endocrine adenoma-peptic ulcer complex . Medicine . 1964;43:481-516.Crossref 8. Shepherd JJ. The natural history of multiple endocrine neoplasia type 1: highly uncommon or highly unrecognized? Arch Surg . 1991;126:935-952.Crossref 9. Aron DC, Findling JW, Fitzgerald PA, et al. Pituitary ACTH dependency of nodular adrenal hyperplasia in Cushing's syndrome: report of two cases and review of the literature . Am J Med . 1981;71:302-306.Crossref 10. Aiba M, Hirayama A, Iri H, et al. Adrenocorticotrophic hormone—independent bilateral adrenocortical macronodular hyperplasia as a distinct subtype of Cushing's syndrome . Am J Clin Pathol . 1991;96:334-340. 11. Lacroix A, Bolte E, Tremblay J, et al. Gastric inhibitory polypeptide-dependent cortisol hypersecretion: a new cause of Cushing's syndrome . N Engl J Med . 1992;327:974-985.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Surgery American Medical Association

Adrenal Lesions in a Large Kindred With Multiple Endocrine Neoplasia Type 1

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Publisher
American Medical Association
Copyright
Copyright © 1996 American Medical Association. All Rights Reserved.
ISSN
0004-0010
eISSN
1538-3644
DOI
10.1001/archsurg.1996.01430190021006
Publisher site
See Article on Publisher Site

Abstract

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 References 1. Wermer P. Genetic aspects of adenomatosis of endocrine glands . Am J Med . 1954;16:363-371.Crossref 2. Bystrom C, Larsson C, Blomberg C, et al. Localisation of the MEN 1 gene to a small region within chromosome 11q13 deletion mapping in tumours . Proc Natl Acad Sci USA . 1990;87:1968-1990.Crossref 3. Knudson AG. Mutation and cancer: statistical study of retinoblastoma . Nature . 1971;332:85-87. 4. Brandi ML, Marx SJ, Aurbach GD, Fitzpatrick LD. Familial multiple endocrine neoplasia type 1: a new look at pathophysiology . Endocr Rev . 1987;8:391-405.Crossref 5. Gross MD, Shapiro B. Clinically silent adrenal masses . J Clin Endocrinol Metab . 1993;77:885-888. 6. Skogseid B, Larsson C, Lindgren P, et al. Clinical and genetic features of adrenocortical lesions in multiple endocrine neoplasia type 1 . J Clin Endocrinol Metab . 1992;75:76-81. 7. Ballard HS, Frame B, Hartsock RJ. Familial multiple endocrine adenoma-peptic ulcer complex . Medicine . 1964;43:481-516.Crossref 8. Shepherd JJ. The natural history of multiple endocrine neoplasia type 1: highly uncommon or highly unrecognized? Arch Surg . 1991;126:935-952.Crossref 9. Aron DC, Findling JW, Fitzgerald PA, et al. Pituitary ACTH dependency of nodular adrenal hyperplasia in Cushing's syndrome: report of two cases and review of the literature . Am J Med . 1981;71:302-306.Crossref 10. Aiba M, Hirayama A, Iri H, et al. Adrenocorticotrophic hormone—independent bilateral adrenocortical macronodular hyperplasia as a distinct subtype of Cushing's syndrome . Am J Clin Pathol . 1991;96:334-340. 11. Lacroix A, Bolte E, Tremblay J, et al. Gastric inhibitory polypeptide-dependent cortisol hypersecretion: a new cause of Cushing's syndrome . N Engl J Med . 1992;327:974-985.Crossref

Journal

Archives of SurgeryAmerican Medical Association

Published: Jul 1, 1996

References