A 71 year old female was referred to Endocrinology Clinic after presenting to Emergency with bitemporal headache and blood pressure 200/80mmHg. She had a history of pregnancy induced hypertension aged 27 and essential hypertension diagnosed aged 40. At age 57, she experienced her first episode of hypertensive urgency; at the time, screening for endocrine causes of hypertension was negative but computed tomography (CT) of the abdomen was undertaken and revealed a 4cm adrenal mass. This was resected and histopathology showed adrenocortical carcinoma (ACC). Post-operatively her hypertension remained well-controlled until her re-presentation in July 2017.
Screening for hyperaldosteronism, hypercortisolaemia and phaeochromocytoma was once again negative. Given the history of ACC, repeat CT abdomen was arranged and surprisingly revealed a left lingular lobe mass. Biopsy of the mass was consistent with metastatic ACC, which prompted further biochemical investigation for a hormonal cause of hypertension. An extended steroid panel showed elevations in progesterone 1.25 nmol/L (<0.32), 17-hydroxyprogesterone 2.6 nmol/L (<2.0), 17-hydroxyprenenolone 33nmol/L (<6.8), dehydroepiandrostenedione-sulphate 4475 nmol/L (27.0-2440 nmol/L), 11-deoxycorticosterone 8.1 nmol/L (<0.57) and 11-deoxycortisol 10.3 nmol/L (<1.0). The solitary metastasis was resected in October 2017 with ensuing normalisation of all hormone levels and blood pressure.
11-deoxycorticosterone (DOC) is a steroid precursor to aldosterone which can bind to and activate the mineralocorticoid receptor. DOC-secreting ACC is an extremely rare cause of secondary hypertension. In the current case, the close temporal relationship between exacerbation of the patient's hypertension and incidence of ACC prompted further investigations which revealed DOC secretion as the cause of hypertension. An understanding of the cause of DOC hypersecretion in ACC, along with improvements in assay techniques, have now enabled the identification of biomarkers of adrenal malignancy which may improve the specificity of preoperative diagnosis of ACC and prevent unnecessary adrenalectomy.