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Endocrine Abstracts (2022) 81 EP106 | DOI: 10.1530/endoabs.81.EP106

ECE2022 Eposter Presentations Adrenal and Cardiovascular Endocrinology (131 abstracts)

11-deoxycorticosterone producing adrenal hyperplasia as a very unusual cause of endocrine hypertension: case report and systematic review of the literature

Queralt Asla Roca 1,2,3 , Helena Sardà Simó 1,2,4 , Enrique Lerma Puertas 2,4,5 , Felicia Alexandra Hanzu 6,7,8 , Eulàlia Urgell Rull 2,9 , José Ignacio Pérez García 2,10 , Susan Webb Youdale 1,2,4,11 & Ana Aulinas Maso 1,2,3,11


1Hospital de la Santa Creu i Sant Pau, Endocrinology and Nutrition, Barcelona, Spain; 2Institut de Recerca – Hospital de la Santa Creu i Sant Pau, Barcelona, Spain; 3University of Vic, Medicine, Vic, Spain; 4Universitat Autònoma de Barcelona, Medicine, Bellaterra, Spain; 5Hospital de la Santa Creu i Sant Pau, Pathological Anatomy, Barcelona, Spain; 6Hospital Clínic, Endocrinology and Nutrition, Barcelona, Spain; 7Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain; 8Universitat de Barcelona, Medicine and Health Sciences, Barcelona, Spain; 9Hospital de la Santa Creu i Sant Pau, Biochemistry, Barcelona, Spain; 10Hospital de la Santa Creu i Sant Pau, General and Digestive Surgery, Barcelona, Spain; 11Carlos III Health Institute, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBER-ER, Unit 747), Madrid, Spain.


11-deoxycorticosterone (DOC) is an aldosterone precursor synthesized from progesterone and converted to corticosterone in the adrenal cortex. DOC overproduction due to an adrenal lesion is a very rare cause of mineralocorticoid-induced hypertension. The objective of this study is to provide the most relevant clinical features that clinicians dealing with patients presenting with the hallmarks of hypertension due to DOC-producing adrenal lesions should be aware of. We report a case of a patient with a DOC producing adrenal hyperplasia and provide a systematic review of all published cases (PubMed, Web of Science) until 2021. A 53-year-old woman without any previous history of hypertension consulted to the Emergency Department for high blood pressure (BP), muscle aches and stiffness. Blood test showed serum potassium of 1.73 mmol/l. After being admitted to the Intensive Care Unit and the hospitalization ward, she was diagnosed of a left functional adrenal mass due to non-aldosterone dependent mineralocorticoid secretion (aldosterone: 81.94 pmol/l; reference range 187–930 and plasma renin activity: undetectable), namely excessive DOC production (35.8 ng/dl; reference range 2–15). A left adrenalectomy was performed. Macroscopy revealed a cortical adenomatous hyperplasia and microscopy excluded atypia, mitosis or necrosis, with a Ki67 index positive in <1% of cells. After surgery, her potassium levels normalized and BP and DOC levels significantly improved. The systematic review of the literature identified 44 cases (including ours). Most cases (30, 68%) affected women with a mean age of 42.8±15.6 years and presented with high BP and hypokalaemia (average: 2.65±0.61 mmol/l). Median (interquartile range) time from onset of first suggestive symptoms to diagnosis was 24 (56) months. DOC levels were a median of 15.8 (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 29 (66%) cases. Carcinoma was the most common histological type (48.8%). Median tumour size was 64 (67.5) mm. Reduced 11β-hydroxylase and 17α-hydroxylase enzyme activities were the most frequent immunohistochemical findings. Malignant compared to benign lesions were larger (97 vs 40 mm, P=0.0001), had higher DOC levels (20.3 vs 6.2 times above the upper limit of normal, P=0.041) and shorter time of evolution (11 vs 42 months, P=0.029). In conclusion, DOC-producing adrenal lesions are very rare, affecting mostly middle-aged women with a primary aldosteronism-like clinical presentation and carcinoma is the most frequent diagnosis. Measuring DOC levels when low aldosterone levels are present in hypertensive patients is advisable.

Volume 81

European Congress of Endocrinology 2022

Milan, Italy
21 May 2022 - 24 May 2022

European Society of Endocrinology 

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