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

Abstract

Background and Objectives11-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.Design and MethodsWe 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.ResultsWe 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.Conclusions11-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.Systematic Review RegistrationOpen Science Framework, 10.17605/OSF.IO/NR7UV.

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“…Deficiency of 11β-hydroxylase disrupts cortisol synthesis, which, through a negative feedback mechanism, stimulates ACTH synthesis and leads to the formation of cortisol and aldosterone precursors, as well as androgens [ 63 ]. Furthermore, 11-deoxycorticosterone has mineralocorticoid activity and its accumulation leads to the development of arterial hypertension, sometimes with a malignant course that cannot be alleviated with medication [ 64 ].…”

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“…Early-onset hypertension is another common feature due to the excess of 11-deoxycorticosterone, which possesses a mild mineralocorticoid action, and is documented as a very rare cause of mineralocorticoid-induced hypertension. In rare cases, electrolyte abnormalities such as hypokalemia may also occur [ 1 , 2 ]. Herein, we present a case of 11βOHD, with all these features.…”