11-Deoxycorticosterone Producing Adrenal Hyperplasia as a Very Unusual Cause of Endocrine Hypertension: Case Report and Systematic Review of the Literature

• Published in: Frontiers in endocrinology (Lausanne) Vol. 13; p. 846865
• Main Authors: Asla, Queralt, Sardà, Helena, Lerma, Enrique, Hanzu, Felicia A, Rodrigo, María Teresa, Urgell, Eulàlia, Pérez, José Ignacio, Webb, Susan M, Aulinas, Anna
• Format: Journal Article
• Language: English
• Published: Switzerland Frontiers Media S.A 31-03-2022
• Subjects: 11-deoxycorticosterone (DOC); Adrenal Gland Neoplasms - complications; Adrenal Gland Neoplasms - diagnosis; adrenal hyperplasia; Adrenal Hyperplasia, Congenital - complications; Adrenal Hyperplasia, Congenital - diagnosis; adrenocortical carcinoma; Adult; Aldosterone; Carcinoma; Desoxycorticosterone; DOC-producing adrenal tumor; Endocrinology; Female; Humans; Hyperplasia - complications; Hypertension; Male; Middle Aged; mineralocorticoid excess; mineralocorticoid hypertension; 11-deoxycorticosterone (DOC); DOC-producing adrenal tumor; adrenal hyperplasia; mineralocorticoid excess; adrenocortical carcinoma; adrenal adenoma; mineralocorticoid hypertension
• ISSN: 1664-2392; 1664-2392
• DOI: 10.3389/fendo.2022.846865

11-deoxycorticosterone overproduction due to an adrenal tumor or hyperplasia is a very rare cause of mineralocorticoid-induced hypertension. The objective is to provide the most relevant clinical features that clinicians dealing with patients presenting with the hallmarks of hypertension due to 11-deoxycorticosterone-producing adrenal lesions should be aware of.
We report the case of a patient with an 11-deoxycorticosterone-producing adrenal lesion and provide a systematic review of all published cases (PubMed, Web of Science and EMBASE) between 1965 and 2021.
We identified 46 cases (including ours). Most cases (31, 67%) affected women with a mean age of 42.9 ± 15.2 years and presented with high blood pressure and hypokalemia (average of 2.68 ± 0.62 mmol/L). Median (interquartile range) time from onset of first suggestive symptoms to diagnosis was 24 (55) months. Aldosterone levels were low or in the reference range in 98% of the cases when available. 11-deoxycorticosterone levels were a median of 12.5 (18.9) times above the upper limit of the normal reference range reported in each article and overproduction of more than one hormone was seen in 31 (67%). Carcinoma was the most common histological type (21, 45.7%). Median tumor size was 61.5 (60) mm. Malignant lesions were larger, had higher 11-deoxycorticosterone levels and shorter time of evolution at diagnosis compared to benign lesions.
11-deoxycorticosterone-producing adrenal lesions are very rare, affecting mostly middle-aged women with a primary aldosteronism-like clinical presentation and carcinoma is the most frequent histological diagnosis. Measuring 11-deoxycorticosterone levels, when low aldosterone levels or in the lower limit of the reference range are present in hypertensive patients, is advisable.
Open Science Framework, 10.17605/OSF.IO/NR7UV.