We describe the case of a 69-year-old woman with bilateral adrenal incidentalomas identified in CT-scan: on the right, a 57 mm heterogeneous mass with <10 Hounsfield units(HU) with absolute washout of 16%; on the left a 13 mm mass with 35UH, intense contrast enhancement but washout of 66%. She had a recent onset of diabetes, hypertension, androgenic alopecia and facial hair. The systolic blood pressure remained persistently >160 mmHg despite receiving four antihypertensive drugs. She referred multiple episodes of syncope in the orthostatic position without palpitations, facial flushing or diaphoresis. She had no malignancy history nor had any apparent syndromic physical features. Her BMI was 32.3 kg/m2, waist circunference of 110 cm with thin members and she presented with androgenic alopecia, submental hair lacking other Cushing features. Hormonal tests showed elevation of plasmatic metanephrines: metanephine 79.9 pg/ml (<60), normetanephrine 214.6 pg/ml (<120); 3-metoxityramin of 12.7 pg/ml (<14); 1 mg dexamethasone suppression test of 4.7 μg/dl with supressed ACTH <5 pg/ml; and elevation of total-testosterone 1.2 ng/ml (<0.90), DHEA-SO4 1.54 μg/ml (0.150.8) and androstenedione 6.7 ng/ml (0.53.4). 17-hydroxyprogesterone: 0.67 ng/ml (0.12.3). 18 F-FDOPA PET confirmed a pheochromocytoma on the left adrenal gland without metastatic lesions. She began treatment with phenoxybenzamine. Gadolinium MRI suggested a adrenocortical carcinoma on the right. Urology proposed right adenomectomy and total adrenalectomy of the left adrenal gland, then converted to total adrenalectomy of the right gland and parcial adrenalectomy of the left because the pheochromocytoma was macroscopically hard to find even with patient mobilization. Histopathology was consistent with a pheochromocytoma on the left adrenal gland (PASS-score 4/20) and adenoma on the right. After surgery there was normalization of the levels of metanephrines and androgens. Synacthen showed a 60 minute cortisol of 10 μg/dl. She was discharged with 10 mg hydrocortisone once a day. The genetic study is in process.
Discussion: There are few reports on bilateral adrenal masses since they are uncommon, especially if from different etiologies. This patient had simultaneously a pheochromocytoma and a cortisol plus androgen-producing adenoma confirmed by normalization of hormones after its resection androgen-hypersecretion by benign tumors is also unusual since is more typical of carcinomas. The surgeon was faced with the need to decide the type of surgical approach at the last minute: there are few recommendations on adrenal surgery but there is general consensus to perform total adrenelectomy on pheochromocytoma-affected glands. However, complete normalization of metanephrines and eventual future adrenocortical-hormone sufficiency are good outcome predictors.
21 May 2022 - 24 May 2022