PMID: 20625655Jul 14, 2010Paper

Giant adrenal myelolipoma associated with 21-hydroxylase deficiency: unusual association mimicking an androgen-secreting adrenocortical carcinoma

Arquivos brasileiros de endocrinologia e metabologia
Lívia Mara MermejoPaula Conde Lamparelli Elias

Abstract

The objective of this study was to describe a case of giant myelolipoma associated with undiagnosed congenital adrenal hyperplasia (CAH) due to 21-hydroxylase (21OH) deficiency. Five seven year-old male patient referred with abdominal ultrasound revealing a left adrenal mass. Biochemical investigation revealed hyperandrogenism and imaging exams characterized a large heterogeneous left adrenal mass with interweaving free fat tissue, compatible with the diagnosis of myelolipoma, and a 1.5 cm nodule in the right adrenal gland. Biochemical correlation has brought concerns about differential diagnosis with adrenocortical carcinoma, and surgical excision of the left adrenal mass was indicated. Anatomopathologic findings revealed a myelolipoma and multinodular hyperplasic adrenocortex. Further investigation resulted in the diagnosis of CAH due to 21OH deficiency. Concluded that CAH has been shown to be associated with adrenocortical tumors. Although rare, myelolipoma associated with CAH should be included in the differential diagnosis of adrenal gland masses. Moreover, CAH should always be ruled out in incidentally detected adrenal masses to avoid unnecessary surgical procedures.

References

May 1, 1979·Clinical Endocrinology·I MaschlerE N Ehrenfeld
Mar 1, 1992·The Journal of Clinical Endocrinology and Metabolism·S JareschR Schlaghecke
Oct 1, 1987·Human Pathology·H SasanoN Sasano
Oct 1, 1995·The Journal of Urology·O UkimuraH Watanabe
Dec 1, 1994·AJR. American Journal of Roentgenology·R F McLoughlin, J H Bilbey
Jun 26, 2001·Endocrine Practice : Official Journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists·C ParenteauP Maheux
Oct 3, 2001·Internal Medicine·T NagaiM Mori
Jan 26, 2002·European Journal of Radiology·Mark E LockhartPhilip J Kenney
May 30, 2002·Archives of Pathology & Laboratory Medicine·Shakil H MerchantPatricia Troncoso
May 15, 2003·Endocrine Pathology·Kimberly H AllisonBrian P Rubin
Oct 28, 2003·Journal of Magnetic Resonance Imaging : JMRI·Nikolaos L KelekisDimitrios A Kelekis
May 19, 2004·International Journal of Urology : Official Journal of the Japanese Urological Association·Francesca ManasseroCesare Selli
May 26, 2005·Archives of Pathology & Laboratory Medicine·Xichun SunClaudia Y Castro
Jul 13, 2006·International Journal of Urology : Official Journal of the Japanese Urological Association·Manabu SakakiHiro-Omi Kanayama
Oct 5, 2007·Endocrine-related Cancer·Ioannis IliasKarel Pacak
Apr 18, 2009·Experimental and Clinical Endocrinology & Diabetes : Official Journal, German Society of Endocrinology [and] German Diabetes Association·C LamasF Botella

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Citations

Feb 8, 2013·Journal of Medical Case Reports·Florbela FerreiraIsabel do Carmo
Feb 22, 2012·Urology Annals·Shahina BanoUmesh Carga Garga
Oct 25, 2012·Case Reports in Pathology·Lea F SurreyMichael D Feldman
Aug 21, 2014·Case Reports in Surgery·S Al-BahriD V Nasrallah
Jun 7, 2015·The Journal of Steroid Biochemistry and Molecular Biology·Adina F Turcu, Richard J Auchus
Jul 17, 2014·Gynecological Endocrinology : the Official Journal of the International Society of Gynecological Endocrinology·Mengte ShiFeixia Shen
Feb 27, 2016·Endocrine Practice : Official Journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists·Henrik Falhammar, David J Torpy
Nov 6, 2014·Arquivos brasileiros de endocrinologia e metabologia·Sara Reis TeixeiraJorge Elias Junior
Dec 4, 2019·Ultrasonography·Christoph F DietrichChristian Jenssen
Feb 18, 2011·Endocrine Practice : Official Journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists·Edgar German-MenaSteven N Levine
Apr 28, 2020·Endocrine Practice : Official Journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists·Ingrid Nermoen, Henrik Falhammar
Jan 21, 2021·Endocrine Practice : Official Journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists·Ingrid Nermoen, Henrik Falhammar

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