An 18-years-old woman without medical history was referred for study of hypertension and an adrenal incidentaloma. The family history was significant for essential well-controlled hypertension in her father; diagnosed at 39 years-old. The patient complained of headache, asthenia, edema in lower limbs, high blood pressure values (200/150 mmHg) for one year (well controlled with losartan 100 mg/12 h, amlodipine 10 mg/24 h and doxazosin 4 mg/24 h) and marked facial acne previous months; without hirsutism. She had regular menstrual cycles. Physical examination: weight 82.4 kg, BMI 28 kg/m2, BP 131/89 mmHg, no acanthosis nigricans and no clinical stigmata of hypercortisolism. Patient provided abdominal CT without contrast with a hypodense nodular 0.8 cm thickening in left adrenal gland, normal renal Doppler ultrasound and echocardiogram with moderate left ventricle eccentric hypertrophy. We requested laboratory tests: glycemia 92 mg/dl, creatinine 0.75 mg/dl, glomerular filtration rate >90 ml/min/1.73 m2, sodium 140 mEq/l, potassium 3.56 mEq/l, TSH 0.88 μUI/ml, FT4 1.1 ng/dl, cortisol 23 mcg/dl, ACTH 11 pg/ml, aldosterone 5.1 ng/dL, PRA 14.9 ng/ml/h, ratio aldosterone/PRA 0.34 (<30), urinary free cortisol 258 μg/24h (<180), dopamine 573 μg/24h (<500), adrenaline 4.4 μg/24h (<35), noradrenaline 140 μg/24h (<100), normetanefrin 508 μg/24h (<500), metanephrines 186 μg/24h (<300) and Nugent test 14 mcg/dl. Due to discordance with initial study, we requested a new study interrupting antihypertensive treatment and administering verapamil 240 mg/24h and doxazosin 8 mg/24h, with result: potassium 2.8 mEq/l (<3.5), ACTH 20 pg/ml, cortisol 29 mcg/dl, PRA 20 ng/ml/h, aldosterone 24 ng/dl, aldosterone/PRA 1 (<30), Liddle test 0.6 mcg/dl and catecholamines and metanephrines in urine in range. Suspecting secondary hyperaldosteronism (reninoma) we requested an abdomen CT with contrast showing a solid, well-defined rounded focal 22 mm. cortical lesion in right kidney lower pole, and subtle 8 mm thickening in left adrenal gland. It is derived to Urology for laparoscopic right partial nephrectomy. Histological examination: well-delimited, not encapsulated tumor, without atypia, necrosis, mitosis, vascular invasion or adjacent renal parenchyma invasion. Immunohistochemistry: CD34 and vimentin intensely positive, with no evidence of immunoexpression for actin, CD117 or CK7, compatible with a juxtaglomerular cell tumor (immunoexpression of CD117/actin are pending in a reference center to confirm the lesion). After surgery, the patient remains normotensive without treatment and with normality on the renin-aldosterone axis (PRA 1.2 ng/ml/h, aldosterone 7.6 ng/dl).
18 - 21 May 2019
European Society of Endocrinology