Paucity of ACTH and Androgen Receptors in Massive Myelolipoma of Congenital Adrenal Hyperplasia Patient: More Insight Into Pathogenesis

Lai Y Wong, Peter Campbell, Takeshi Usui, Anthony J O'Sullivan


    Adrenal myelolipomas in congenital adrenal hyperplasia (CAH) are rare. The pathogenesis is unknown but adrenocorticotropic hormone (ACTH) is implicated to play a key role. Demonstration of paucity of ACTH and androgen receptors in these tumors may suggest otherwise. We describe a 54-year-old woman, presenting with bilateral massive myelolipoma associated with 21-hydroxylase deficiency CAH. Bilateral adrenalectomy was performed due to significant mass effect. Reverse transcriptase-polymerase chain reaction performed on tumor tissue showed no expression of the ACTH (MC2R - melanocortin 2 receptor) and androgen receptors. There are few published reports on pathogenesis of myelolipoma or in association with CAH. Recent evidence of chromosomal abnormality similar to hematopoietic cancer and inactivation of X chromosome in some myelolipomas suggestive of clonal proliferation from a common pluripotent stem cell has been found. However, origin of the hematopoietic tissue and the triggering factor for proliferation and development of the myelolipomas remain unclear. Nevertheless, the implication of these findings is exciting, as it suggests that development and proliferation of myelolipoma is not necessarily attributable to excess ACTH stimulation alone. Presence of local and systemic growth factors is possibly yet to be determined.



Adrenal; Myelolipoma; Pathogenesis

Full Text: HTML PDF
Home     |     Log In     |      About     |      Search     |      Current     |      Archives     |      Submit      |     Subscribe



Aims and Scope

Current Issues

Conflict of Interest

About Publisher

Editorial Board



Company Profile

Editorial Office

Misconduct and Retraction


Company Registration

Contact Us

Abstracting and Indexing



Instructions to Authors


Declaration of Helsinki

Contact Publisher

Submission Checklist


Terms of Use

Company Address

Submit a Manuscript

Open Access Policy

Privacy Policy

Browse Journals

Publishing Fee

Publishing Policy


Recent Highlights

Peer-Review Process

Publishing Quality

Code of Ethics

Advertising Policy

Manuscript Tracking

Advanced Search

For Librarians


Publishing Process

Publication Frequency

For Reviewers

Propose a New Journal


Journal of Endocrinology and Metabolism, bimonthly, ISSN 1923-2861 (print), 1923-287X (online), published by Elmer Press Inc.        
The content of this site is intended for health care professionals.
This is an open-access journal distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License, which permits unrestricted
non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Creative Commons Attribution license (Attribution-NonCommercial 4.0 International CC-BY-NC 4.0)

This journal follows the International Committee of Medical Journal Editors (ICMJE) recommendations for manuscripts submitted to biomedical journals,
the Committee on Publication Ethics (COPE) guidelines, and the Principles of Transparency and Best Practice in Scholarly Publishing.

website:   editorial contact:
Address: 9225 Leslie Street, Suite 201, Richmond Hill, Ontario, L4B 3H6, Canada

© Elmer Press Inc. All Rights Reserved.