Virilizing Adrenocortical Carcinoma: Development in a Patient With Salt-Losing Congenital Adrenal Hyperplasia
Abstract
CONGENITAL adrenal hyperplasia (CAH) is an uncommon disorder, but the incidence of virilizing adrenocortical neoplasms, benign and malignant, is even less common.1The occurrence of both conditions in the same patient2-5raises the question of whether or not the association is coincidental. The patient described herein was born with a salt-losing form of CAH, probably caused by a severe 21-hydroxylase deficiency; despite adequate control, a virilizing carcinoma of the adrenal cortex developed. Report of a Case An 11-year-old girl was admitted to the White Plains (NY) Hospital Medical Center because of progressive virilization associated with strikingly increasing levels of urinary 17-ketosteroids (17-KS). During the first week of life, the patient had had persistent vomiting, weakness, and convulsions. Serum sodium level had been 126 mEq/L, and serum potassium level, 9.2 mEq/L. The clitoris had been enlarged and the labia majora fused. A cystourethrogram had shown dye in a vaginal
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Some cases of ACC in conjunction with congenital adrenal hyperplasia (CAH) have been described.
“Some cases of ACC in conjunction with congenital adrenal hyperplasia (CAH) have been described (75).”
From Adrenocortical carcinoma (2014) · cites this paper as [75]
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References 6 · Cited by 56
- Authors
- Bauman, A.
- Corresponding author
- Bauman, Arthur
- Venue
- JAMA
- Publisher
- American Medical Association (AMA)
- Published
- 1982-12-17
- Volume
- 248
- Issue
- 23
- Pages
- 3140
- ISSN
- 0002-9955 (ISSN-L)
- 0098-7484
- OpenAlex
- W2010945407
- Cited by
- 56
- References
- 6
- Citations by year
- 2024: 2 · 2020: 1 · 2018: 1 · 2017: 2 · 2016: 2 · 2015: 1 · 2014: 1 · 2013: 4
- FWCI
- 1.29
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- JAMA (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Sexual Differentiation and Disorders
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
- Keywords
- congenital adrenal hyperplasia, 21-hydroxylase deficiency, salt-losing congenital adrenal hyperplasia, adrenal cortex neoplasms, masculinization, urinary 17-ketosteroids, pediatric patients, clitoromegaly, hypokalemia, hyponatremia
- MeSH terms
- Adrenal Cortex Neoplasms, Adrenal Glands, Adrenal Hyperplasia, Congenital, Atrophy, Carcinoma, Child, Female, Humans, Virilism, 17-Ketosteroids
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