Endocrine Abstracts

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.