11-Deoxycorticosterone Producing Adrenal Hyperplasia as a Very Unusual Cause of Endocrine Hypertension : Case Report and Systematic Review of the Literature
Asla, Queralt (Universitat de Vic)
Sardà, Helena (Universitat Autònoma de Barcelona. Departament de Medicina)
Lerma Puertas, Enrique (Institut d'Investigació Biomèdica Sant Pau)
Hanzu, Felicia A. (Universitat de Barcelona)
Rodrigo, María Teresa (Hospital Clínic i Provincial de Barcelona)
Urgell, Eulàlia (Institut d'Investigació Biomèdica Sant Pau)
Pérez, José Ignacio (Institut d'Investigació Biomèdica Sant Pau)
Webb, S. M 1952- (Centro de Investigación Biomédica en Red de Enfermedades Raras)
Aulinas, Anna (Centro de Investigación Biomédica en Red de Enfermedades Raras)

Data:	2022
Resum:	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.
Drets:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.
Llengua:	Anglès
Document:	Article de revisió ; recerca ; Versió publicada
Matèria:	Mineralocorticoid hypertension ; Mineralocorticoid excess ; 11-deoxycorticosterone (DOC) ; DOC-producing adrenal tumor ; Adrenal hyperplasia ; Adrenocortical carcinoma ; Adrenal adenoma
Publicat a:	Frontiers in endocrinology, Vol. 13 (march 2022) , ISSN 1664-2392

DOI: 10.3389/fendo.2022.846865
PMID: 35432204

15 p, 4.1 MB

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