A Gigantic Uterine Leiomyoma and Big Bilateral Adrenal Myelolipomas as a Result of Untreated Congenital Adrenal Hyperplasia

Introduction: Patients with congenital adrenal hyperplasia (CAH) can present early with salt wasting, adrenal insufficiency, and hyperandrogenism. Late consequences as a result of untreated CAH are now rarely seen. We present a patient with a massive uterine leiomyoma and bilateral adrenal myelolipo...

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Autores principales:	Betty La, MD, Celestine Tung, MD, Eugene A. Choi, MD, Ha Nguyen, MD
Formato:	article
Lenguaje:	EN
Publicado:	Elsevier 2021
Materias:	adrenal myelolipoma congenital adrenal hyperplasia uterine leiomyoma Diseases of the endocrine glands. Clinical endocrinology RC648-665
Acceso en línea:	https://doaj.org/article/502b050aa24d4ca8aa230466281f13f3
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spelling	oai:doaj.org-article:502b050aa24d4ca8aa230466281f13f32021-11-06T04:32:34ZA Gigantic Uterine Leiomyoma and Big Bilateral Adrenal Myelolipomas as a Result of Untreated Congenital Adrenal Hyperplasia2376-060510.1016/j.aace.2021.05.002https://doaj.org/article/502b050aa24d4ca8aa230466281f13f32021-11-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S237606052100064Xhttps://doaj.org/toc/2376-0605Introduction: Patients with congenital adrenal hyperplasia (CAH) can present early with salt wasting, adrenal insufficiency, and hyperandrogenism. Late consequences as a result of untreated CAH are now rarely seen. We present a patient with a massive uterine leiomyoma and bilateral adrenal myelolipomas due to longstanding treatment noncompliance. Case Report: A female patient with CAH was treated with glucocorticoids until the age of 29 years when they stopped with the intention of identifying as a male. The patient then presented with abdominal pain and distension. Computed tomography images of the abdomen and pelvis revealed a 31 × 35 × 31-cm abdominal mass, a 5.9× 2.4-cm right adrenal mass, and an 11.8 × 8.8-cm left adrenal mass. The patient underwent total hysterectomy and bilateral adrenalectomy. Pathology of the abdominal mass was consistent with uterine leiomyoma, and bilateral adrenal masses were consistent with adrenal myelolipomas. Discussion: The goal of CAH therapy is to provide adequate replacement while reducing adrenocorticotropic hormone and adrenal androgens levels. Due to the conversion of androgens to estrogens, untreated females with CAH have elevated androgen and estrogen levels. High levels of these hormones can stimulate the growth of estrogen-dependent organs as exemplified by our patient. Chronic adrenocorticotropic hormone stimulation can not only cause adrenal hyperplasia but has also been associated with the development of adrenal myelolipomas. Conclusion: This case demonstrates the significance of CAH treatment compliance as there are several serious sequela outside of the expected adrenal insufficiency and virilization. Even when the desired effect is virilization, other means of hormonal therapy should be considered.Betty La, MDCelestine Tung, MDEugene A. Choi, MDHa Nguyen, MDElsevierarticleadrenal myelolipomacongenital adrenal hyperplasiauterine leiomyomaDiseases of the endocrine glands. Clinical endocrinologyRC648-665ENAACE Clinical Case Reports, Vol 7, Iss 6, Pp 342-345 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	adrenal myelolipoma congenital adrenal hyperplasia uterine leiomyoma Diseases of the endocrine glands. Clinical endocrinology RC648-665
spellingShingle	adrenal myelolipoma congenital adrenal hyperplasia uterine leiomyoma Diseases of the endocrine glands. Clinical endocrinology RC648-665 Betty La, MD Celestine Tung, MD Eugene A. Choi, MD Ha Nguyen, MD A Gigantic Uterine Leiomyoma and Big Bilateral Adrenal Myelolipomas as a Result of Untreated Congenital Adrenal Hyperplasia
description	Introduction: Patients with congenital adrenal hyperplasia (CAH) can present early with salt wasting, adrenal insufficiency, and hyperandrogenism. Late consequences as a result of untreated CAH are now rarely seen. We present a patient with a massive uterine leiomyoma and bilateral adrenal myelolipomas due to longstanding treatment noncompliance. Case Report: A female patient with CAH was treated with glucocorticoids until the age of 29 years when they stopped with the intention of identifying as a male. The patient then presented with abdominal pain and distension. Computed tomography images of the abdomen and pelvis revealed a 31 × 35 × 31-cm abdominal mass, a 5.9× 2.4-cm right adrenal mass, and an 11.8 × 8.8-cm left adrenal mass. The patient underwent total hysterectomy and bilateral adrenalectomy. Pathology of the abdominal mass was consistent with uterine leiomyoma, and bilateral adrenal masses were consistent with adrenal myelolipomas. Discussion: The goal of CAH therapy is to provide adequate replacement while reducing adrenocorticotropic hormone and adrenal androgens levels. Due to the conversion of androgens to estrogens, untreated females with CAH have elevated androgen and estrogen levels. High levels of these hormones can stimulate the growth of estrogen-dependent organs as exemplified by our patient. Chronic adrenocorticotropic hormone stimulation can not only cause adrenal hyperplasia but has also been associated with the development of adrenal myelolipomas. Conclusion: This case demonstrates the significance of CAH treatment compliance as there are several serious sequela outside of the expected adrenal insufficiency and virilization. Even when the desired effect is virilization, other means of hormonal therapy should be considered.
format	article
author	Betty La, MD Celestine Tung, MD Eugene A. Choi, MD Ha Nguyen, MD
author_facet	Betty La, MD Celestine Tung, MD Eugene A. Choi, MD Ha Nguyen, MD
author_sort	Betty La, MD
title	A Gigantic Uterine Leiomyoma and Big Bilateral Adrenal Myelolipomas as a Result of Untreated Congenital Adrenal Hyperplasia
title_short	A Gigantic Uterine Leiomyoma and Big Bilateral Adrenal Myelolipomas as a Result of Untreated Congenital Adrenal Hyperplasia
title_full	A Gigantic Uterine Leiomyoma and Big Bilateral Adrenal Myelolipomas as a Result of Untreated Congenital Adrenal Hyperplasia
title_fullStr	A Gigantic Uterine Leiomyoma and Big Bilateral Adrenal Myelolipomas as a Result of Untreated Congenital Adrenal Hyperplasia
title_full_unstemmed	A Gigantic Uterine Leiomyoma and Big Bilateral Adrenal Myelolipomas as a Result of Untreated Congenital Adrenal Hyperplasia
title_sort	gigantic uterine leiomyoma and big bilateral adrenal myelolipomas as a result of untreated congenital adrenal hyperplasia
publisher	Elsevier
publishDate	2021
url	https://doaj.org/article/502b050aa24d4ca8aa230466281f13f3
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A Gigantic Uterine Leiomyoma and Big Bilateral Adrenal Myelolipomas as a Result of Untreated Congenital Adrenal Hyperplasia

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