Giant Bilateral Adrenal Myelolipoma with Congenital Adrenal Hyperplasia

Myelolipomas are rare and benign neoplasms, predominant of the adrenal glands, consisting of adipose and mature hematopoietic tissue, commonly discovered incidentally with increased use of radiologic imaging. Few cases of giant bilateral adrenal masses are reported, especially in the setting of cong...

Full description

Saved in:
Bibliographic Details
Main Authors: S. Al-Bahri, A. Tariq, B. Lowentritt, D. V. Nasrallah
Format: Article
Language:English
Published: Wiley 2014-01-01
Series:Case Reports in Surgery
Online Access:http://dx.doi.org/10.1155/2014/728198
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1832556997682135040
author S. Al-Bahri
A. Tariq
B. Lowentritt
D. V. Nasrallah
author_facet S. Al-Bahri
A. Tariq
B. Lowentritt
D. V. Nasrallah
author_sort S. Al-Bahri
collection DOAJ
description Myelolipomas are rare and benign neoplasms, predominant of the adrenal glands, consisting of adipose and mature hematopoietic tissue, commonly discovered incidentally with increased use of radiologic imaging. Few cases of giant bilateral adrenal masses are reported, especially in the setting of congenital adrenal hyperplasia (CAH). We report the case of a 39-year-old male with a history of CAH secondary to 21-α hydroxylase deficiency on steroids since childhood, self-discontinued during adolescence, presenting with abdominal distension, fatigue, decreased libido, and easy bruising. Imaging revealed giant bilateral adrenal masses. He subsequently underwent bilateral adrenalectomy found to be myelolipomas measuring 30 × 25 × 20 cm on the left and weighing 4.1 kg and 25 × 20 × 13 cm on the right and weighing 2.7 kg. Adrenal myelolipomas are found to coexist with many other conditions such as Cushing’s syndrome, Addison’s disease, and CAH. We discuss the association with high adrenocorticotropic hormone (ACTH) states and review the studies involving ACTH as proponent leading to myelolipomas. Massive growth of these tumors, as in our case, can produce compression and hemorrhagic symptoms. We believe it is possible that self-discontinuation of steroids, in the setting of CAH, may have resulted in the growth of his adrenal masses.
format Article
id doaj-art-f6c654cda05f4bf199c9b43dd9d24f30
institution Kabale University
issn 2090-6900
2090-6919
language English
publishDate 2014-01-01
publisher Wiley
record_format Article
series Case Reports in Surgery
spelling doaj-art-f6c654cda05f4bf199c9b43dd9d24f302025-02-03T05:43:55ZengWileyCase Reports in Surgery2090-69002090-69192014-01-01201410.1155/2014/728198728198Giant Bilateral Adrenal Myelolipoma with Congenital Adrenal HyperplasiaS. Al-Bahri0A. Tariq1B. Lowentritt2D. V. Nasrallah3Department of General Surgery, MedStar Union Memorial Hospital, Suite 655 B, Johnston Professional Building, 201 East University Parkway, Baltimore, MD 21218, USASchool of Medicine, Saba University, 27 Jackson Road, Suite 301, Devens, MA 01434, USADepartment of General Surgery, MedStar Union Memorial Hospital, Suite 655 B, Johnston Professional Building, 201 East University Parkway, Baltimore, MD 21218, USADepartment of General Surgery, MedStar Union Memorial Hospital, Suite 655 B, Johnston Professional Building, 201 East University Parkway, Baltimore, MD 21218, USAMyelolipomas are rare and benign neoplasms, predominant of the adrenal glands, consisting of adipose and mature hematopoietic tissue, commonly discovered incidentally with increased use of radiologic imaging. Few cases of giant bilateral adrenal masses are reported, especially in the setting of congenital adrenal hyperplasia (CAH). We report the case of a 39-year-old male with a history of CAH secondary to 21-α hydroxylase deficiency on steroids since childhood, self-discontinued during adolescence, presenting with abdominal distension, fatigue, decreased libido, and easy bruising. Imaging revealed giant bilateral adrenal masses. He subsequently underwent bilateral adrenalectomy found to be myelolipomas measuring 30 × 25 × 20 cm on the left and weighing 4.1 kg and 25 × 20 × 13 cm on the right and weighing 2.7 kg. Adrenal myelolipomas are found to coexist with many other conditions such as Cushing’s syndrome, Addison’s disease, and CAH. We discuss the association with high adrenocorticotropic hormone (ACTH) states and review the studies involving ACTH as proponent leading to myelolipomas. Massive growth of these tumors, as in our case, can produce compression and hemorrhagic symptoms. We believe it is possible that self-discontinuation of steroids, in the setting of CAH, may have resulted in the growth of his adrenal masses.http://dx.doi.org/10.1155/2014/728198
spellingShingle S. Al-Bahri
A. Tariq
B. Lowentritt
D. V. Nasrallah
Giant Bilateral Adrenal Myelolipoma with Congenital Adrenal Hyperplasia
Case Reports in Surgery
title Giant Bilateral Adrenal Myelolipoma with Congenital Adrenal Hyperplasia
title_full Giant Bilateral Adrenal Myelolipoma with Congenital Adrenal Hyperplasia
title_fullStr Giant Bilateral Adrenal Myelolipoma with Congenital Adrenal Hyperplasia
title_full_unstemmed Giant Bilateral Adrenal Myelolipoma with Congenital Adrenal Hyperplasia
title_short Giant Bilateral Adrenal Myelolipoma with Congenital Adrenal Hyperplasia
title_sort giant bilateral adrenal myelolipoma with congenital adrenal hyperplasia
url http://dx.doi.org/10.1155/2014/728198
work_keys_str_mv AT salbahri giantbilateraladrenalmyelolipomawithcongenitaladrenalhyperplasia
AT atariq giantbilateraladrenalmyelolipomawithcongenitaladrenalhyperplasia
AT blowentritt giantbilateraladrenalmyelolipomawithcongenitaladrenalhyperplasia
AT dvnasrallah giantbilateraladrenalmyelolipomawithcongenitaladrenalhyperplasia