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Pathol Res Pract. 2012 Mar 15;208(3):189-94. doi: 10.1016/j.prp.2011.12.011. Epub 2012 Feb 5.

A unique case of synchronous functional adrenocortical adenoma and myelolipoma within the ectopic adrenal cortex in a child with Beckwith-Wiedemann syndrome.

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  • 1Department of Pathology, Boldrini Children's Hospital, Campinas, SP, Brazil. izilda@boldrini.org.br

Abstract

We report a unique case of synchronous functional adrenocortical adenoma and an incidental myelolipoma within ectopic cortical adrenal tissue located in the renal hilum in a child with Beckwith-Wiedemann syndrome and review the association between adrenal gland disorders and myelolipomas. To the best of our knowledge, this is the first documented case of a simultaneous occurrence of these three conditions. A 17-month-old child with Beckwith-Wiedemann syndrome was diagnosed with a left adrenal tumor during complementary radiologic studies. Biochemical investigation before surgery showed elevated blood levels of cortisol and dehydroepiandrosterone hormones. The patient underwent a left adrenalectomy with ipsilateral renal hilar and intercaval-aortic lymph node dissection. Pathology findings revealed a left adrenocortical adenoma and an incidental myelolipoma growing within ectopic cortical adrenal tissue in the renal hilum. The patient is doing well and does not have any current health issues. Patients with adrenal cortex disorders, such as hyperplasias and neoplasms, particularly when associated with hormonal imbalances, may have an increased risk of developing myelolipomas. Whether Beckwith-Wiedemann syndrome may, by itself, contribute to simultaneous occurrence of adrenocortical adenomas and myelolipomas remains to be clarified.

Copyright © 2012 Elsevier GmbH. All rights reserved.

PMID:
22309953
[PubMed - indexed for MEDLINE]
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