Document Detail


Adrenal Myelolipomas in Patients with Congenital Adrenal Hyperplasia: Review of the Literature and a Case Report.
MedLine Citation:
PMID:  21324823     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Objective: To review the association between congenital adrenal hyperplasia and adrenal myelolipomas and report a case of bilateral, giant adrenal myelolipomas in a patient with untreated congenital adrenal hyperplasia due to 21-hydroxylase deficiency.Methods: We present a case report and a review of the relevant English literature concerning patients diagnosed with both congenital adrenal hyperplasia and myelolipomas.Results: A 45 year old white man with untreated 21-hydroxylase deficiency congenital adrenal hyperplasia presented with increasing abdominal girth and abdominal pain. CT of the abdomen demonstrated 22 × 11 cm left and 6 × 5.5 cm right adrenal masses that were of very low density consistent with adrenal myelolipomas. He had resection of a 24.4 × 19.0 × 9.5 cm left adrenal myelolipoma weighing 2556.8 grams. The mass was composed of mature adipose tissue with areas of hematopoietic cells of myeloid, erythroid, and megakaryocytic cell lines. Islands of adrenal cortical cells were scattered between the adipose and hematopoietic tissue. Including the present case, we could identify 31 patients with both congenital adrenal hyperplasia and myelolipomas that have been reported in the English literature. The details of these patients are reviewed. Conclusion: There may be an increased risk of adrenal myelolipomas developing in patients with congenital adrenal hyperplasia, particularly if it is poorly controlled. How, and whether, chronic exposure of the adrenal glands to high levels of ACTH increases the risk of developing myelolipomas remains a matter of speculation.
Authors:
Edgar German-Mena; Gazi B Zibari; Steven N Levine
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-2-16
Journal Detail:
Title:  Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists     Volume:  -     ISSN:  1934-2403     ISO Abbreviation:  -     Publication Date:  2011 Feb 
Date Detail:
Created Date:  2011-2-17     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9607439     Medline TA:  Endocr Pract     Country:  -    
Other Details:
Languages:  ENG     Pagination:  1-25     Citation Subset:  -    
Affiliation:
Section of Endocrinology and Metabolism, Department of Internal Medicine, Louisiana State University Health Sciences Center, Shreveport.
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