Secondary endocrine hypertension affects around 10% of all hypertensive population, most frequently primary aldosteronism (PA). Less frequent forms of adrenal hypertension include pheochromocytomas and other causes of mineralocorticoid excess than PA, featuring suppressed renin without excess of aldosterone. Featuring. We present a 29-year-old woman with difficult to control hypertension diagnosed two years before and poor response to ACEI and ARA II treatment. She was referred for study and treatment. She had a normal appearance, without Cushings stigmata, overweight nor clinical suspect of pheochromocytomas. She had not noticed hirsutism and maintained regular menstrual cycles. Biochemical study was normal except for slightly lower potassium (3.48 mmol/l). Hormonal study showed suppressed renin activity (<0.2 ng/ml per h), with normal to low aldosterone (7.3 ng/dl), normal urinary metanephrines and normal plasma ACTH (34.5 pg/ml). Abdominal CT revealed a well-defined heterogeneous and much vascularized right adrenal mass of 6 cm in diameter. Deoxycorticosterone (DOC) measured in previous stored sample was 237.9 ng/dl (normal < 15 ng/dl) with normal deoxycortisol (4.5 ng/ml; NV < 7.2), testosterone, DHEAs and plasmatic and urinary cortisol. Open right adrenalectomy was performed due to the size and suspicion of malignancy. On light microscopic examination, the tumour was an adenoma of 6.5 cm not encapsulated, composed of cells with clear and eosinophilic cytoplasm and large nuclei without mitosis nor necrosis. Weisss criteria classified it as adrenocortical adenoma. Postoperatively, DOC level fell to 7.1 ng/dl and blood pressure and potassium normalized.
Conclusion: DOCA-producing adrenal neoplasm are exceptionally reported, and they are usually malignant tumours. They should be suspected in the presence of adrenal tumours with supressed renin but inappropriate low aldosterone, not suggesting PA. Early diagnosis can be very important because malignancy is the rule in this peculiar pathology.
20 May 2017 - 23 May 2017