Two Adults with Adrenal Myelolipoma and 21-Hydroxylase Deficiency
We present incidentally discovered adrenal myelolipomas in two adult males with untreated congenital adrenal hyperplasia (CAH). The patients had simple virilizing form of CAH due to mutations in the CYP21 gene coding for 21-hydroxylase; one was heterozygous for the I172N mutation and the other compo...
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Format: | Article |
Language: | English |
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Wiley
2009-01-01
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Series: | Case Reports in Medicine |
Online Access: | http://dx.doi.org/10.1155/2009/916891 |
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author | Ingrid Nermoen Ivar Følling Kjetil Vegge Arne Larmo Bjørn Gunnar Nedrebø Eystein Sverre Husebye Kristian Løvås |
author_facet | Ingrid Nermoen Ivar Følling Kjetil Vegge Arne Larmo Bjørn Gunnar Nedrebø Eystein Sverre Husebye Kristian Løvås |
author_sort | Ingrid Nermoen |
collection | DOAJ |
description | We present incidentally discovered adrenal myelolipomas in two adult males with untreated congenital adrenal hyperplasia (CAH). The patients had simple virilizing form of CAH due to mutations in the CYP21 gene coding for 21-hydroxylase; one was heterozygous for the I172N mutation and the other compound heterozygous for the I172N and I2splice mutations. The masses were not removed since myelolipomas are considered benign tumors, and the tumor size did not increase during four- and nine-year observation periods. An adrenal myelolipoma is an important exception to the rule that large tumours should be removed. Untreated CAH with prolonged excessive ACTH stimulation might contribute to the growth of adrenal masses. CAH should be considered as a differential diagnosis of patients with adrenal masses or adrenal myelolipomas. |
format | Article |
id | doaj-art-6cf59dfcc98740e9a424048361bdeb39 |
institution | Kabale University |
issn | 1687-9627 1687-9635 |
language | English |
publishDate | 2009-01-01 |
publisher | Wiley |
record_format | Article |
series | Case Reports in Medicine |
spelling | doaj-art-6cf59dfcc98740e9a424048361bdeb392025-02-03T01:13:12ZengWileyCase Reports in Medicine1687-96271687-96352009-01-01200910.1155/2009/916891916891Two Adults with Adrenal Myelolipoma and 21-Hydroxylase DeficiencyIngrid Nermoen0Ivar Følling1Kjetil Vegge2Arne Larmo3Bjørn Gunnar Nedrebø4Eystein Sverre Husebye5Kristian Løvås6Department of Endocrinology, Akershus University Hospital, 1478 Lørenskog, NorwayDepartment of Endocrinology, Akershus University Hospital, 1478 Lørenskog, NorwayDepartment of Radiology, Akershus University Hospital, 1478 Lørenskog, NorwayDepartment of Radiology, Akershus University Hospital, 1478 Lørenskog, NorwayDepartment of Medicine, Haukeland University Hospital, 5021 Bergen, NorwayDepartment of Medicine, Haukeland University Hospital, 5021 Bergen, NorwayDepartment of Medicine, Haukeland University Hospital, 5021 Bergen, NorwayWe present incidentally discovered adrenal myelolipomas in two adult males with untreated congenital adrenal hyperplasia (CAH). The patients had simple virilizing form of CAH due to mutations in the CYP21 gene coding for 21-hydroxylase; one was heterozygous for the I172N mutation and the other compound heterozygous for the I172N and I2splice mutations. The masses were not removed since myelolipomas are considered benign tumors, and the tumor size did not increase during four- and nine-year observation periods. An adrenal myelolipoma is an important exception to the rule that large tumours should be removed. Untreated CAH with prolonged excessive ACTH stimulation might contribute to the growth of adrenal masses. CAH should be considered as a differential diagnosis of patients with adrenal masses or adrenal myelolipomas.http://dx.doi.org/10.1155/2009/916891 |
spellingShingle | Ingrid Nermoen Ivar Følling Kjetil Vegge Arne Larmo Bjørn Gunnar Nedrebø Eystein Sverre Husebye Kristian Løvås Two Adults with Adrenal Myelolipoma and 21-Hydroxylase Deficiency Case Reports in Medicine |
title | Two Adults with Adrenal Myelolipoma and 21-Hydroxylase Deficiency |
title_full | Two Adults with Adrenal Myelolipoma and 21-Hydroxylase Deficiency |
title_fullStr | Two Adults with Adrenal Myelolipoma and 21-Hydroxylase Deficiency |
title_full_unstemmed | Two Adults with Adrenal Myelolipoma and 21-Hydroxylase Deficiency |
title_short | Two Adults with Adrenal Myelolipoma and 21-Hydroxylase Deficiency |
title_sort | two adults with adrenal myelolipoma and 21 hydroxylase deficiency |
url | http://dx.doi.org/10.1155/2009/916891 |
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