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La B, Tung C, Choi EA, et al. A Gigantic Uterine Leiomyoma and Big Bilateral Adrenal Myelolipomas as a Result of Untreated Congenital Adrenal Hyperplasia. AACE Clin Case Rep. 2021;7(6):342-345doi: 10.1016/j.aace.2021.05.002.
La, B., Tung, C., Choi, E. A., & Nguyen, H. (2021). A Gigantic Uterine Leiomyoma and Big Bilateral Adrenal Myelolipomas as a Result of Untreated Congenital Adrenal Hyperplasia. AACE clinical case reports, 7(6), 342-345. https://doi.org/10.1016/j.aace.2021.05.002
La, Betty, et al. "A Gigantic Uterine Leiomyoma and Big Bilateral Adrenal Myelolipomas as a Result of Untreated Congenital Adrenal Hyperplasia." AACE clinical case reports vol. 7,6 (2021): 342-345. doi: https://doi.org/10.1016/j.aace.2021.05.002
La B, Tung C, Choi EA, Nguyen H. A Gigantic Uterine Leiomyoma and Big Bilateral Adrenal Myelolipomas as a Result of Untreated Congenital Adrenal Hyperplasia. AACE Clin Case Rep. 2021 May 13;7(6):342-345. doi: 10.1016/j.aace.2021.05.002. eCollection 2021. PMID: 34765728; PMCID: PMC8573279.
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AACE Clin Case Rep. 2021 May 13;7(6):342-345. doi: 10.1016/j.aace.2021.05.002. eCollection 2021.

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

AACE clinical case reports

Betty La, Celestine Tung, Eugene A Choi, Ha Nguyen

Affiliations

  1. Section of Endocrinology, Diabetes and Metabolism, Baylor College of Medicine, Houston, Texas.
  2. Division of Gynecologic Oncology, Baylor College of Medicine, Houston, Texas.
  3. Division of Surgical Oncology, Baylor College of Medicine, Houston, Texas.

PMID: 34765728 PMCID: PMC8573279 DOI: 10.1016/j.aace.2021.05.002

Abstract

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.

Keywords: 17-OHP, 17-hydroxyprogesterone; ACTH, adrenocorticotropic hormone; CAH, congenital adrenal hyperplasia; adrenal myelolipoma; congenital adrenal hyperplasia; uterine leiomyoma

References

  1. Surg Endosc. 2008 Feb;22(2):522-6 - PubMed
  2. Horm Res. 2008;69(4):203-11 - PubMed
  3. J Med Case Rep. 2013 Feb 06;7:39 - PubMed
  4. Obstet Gynecol. 1970 Feb;35(2):255-9 - PubMed
  5. J Clin Endocrinol Metab. 2018 May 1;103(5):1767-1778 - PubMed
  6. Endocrine. 2018 Jan;59(1):7-15 - PubMed
  7. J Clin Endocrinol Metab. 2018 Nov 1;103(11):4043-4088 - PubMed
  8. Endocr Pract. 2020 Nov;26(11):1351-1365 - PubMed
  9. J Clin Endocrinol Metab. 2007 Jun;92(6):2169-75 - PubMed
  10. Schweiz Med Wochenschr. 1973 Dec 8;103(49):1743-9 - PubMed
  11. Endocr Rev. 2000 Jun;21(3):245-91 - PubMed
  12. Endocrinology. 2015 Oct;156(10):3504-10 - PubMed
  13. Arch Pediatr Adolesc Med. 1999 Dec;153(12):1272-8 - PubMed
  14. Best Pract Res Clin Endocrinol Metab. 2012 Aug;26(4):405-19 - PubMed
  15. Fertil Steril. 1988 Feb;49(2):224-8 - PubMed
  16. J Gastroenterol Hepatol. 2004 Jul;19(7):826 - PubMed
  17. J Clin Endocrinol Metab. 1992 Mar;74(3):685-9 - PubMed
  18. Endocr Rev. 2020 Dec 1;41(6): - PubMed
  19. Urol J. 2006 Spring;3(2):71-4 - PubMed
  20. J Clin Endocrinol Metab. 2016 Jan;101(1):123-30 - PubMed

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