Endocrine Abstracts

Introduction

Bilateral adrenal incidentalomas represent about 15% of adrenal incidentalomas. Their investigation and management are less discussed and more challenging than unilateral incidentalome. A special attention is required because they are more likely to be pathologic. When associated with a synchronous extra-adrenal tumor, the first diagnosis is adrenal metastasis, but this is not always true. Here we present a rare association of pheochromocytoma, contralateral nonfunctioning adrenal adenoma, and renal angiomyolipoma.

Case presentation

A 69-year-old female, with a history of resistant hypertension, diabetes mellitus and hypercholesterolemia was admitted in our endocrine department for exploration of bilateral adrenal incidentalomas associated with a left renal tumor. She had no family history of endocrine neoplasms. On physical examination, there was no goiter or thyroid nodules. Adrenal insufficiency was ruled out by a normal synachten test. Routine laboratory testing were normal except uncontrolled diabetes. Hormonal investigations showed a high level of urinary fractionated metanephrines (2.3 fold above the upper limit of normal). Basal cortisol level, overnight dexamethasone suppression test, and aldosterone renin ratio were normal. Contrast enhanced computed tomography had shown a typical angiomyolipoma in the upper pole of the left kidney with two adrenal gland masses measuring at right 37 mm with a density of 38 HU and at left 15 mm with a low density (–3 HU). SPECT/CT Metaiodobenzylguanidine (mIBG) scintigraphy was performed and had detected abnormal fixation in the right gland. After 10 days of preoperative preparation, the patient underwent successful right adrenalectomy and the histopathologic features were consistent with pheochromocytoma. After surgery, urinary fractionated metanephrine level was normal and the hypertension was controlled with only monotherapy.

Conclusion

Bilateral adrenal tumors require a meticulous work-up, including hormonal investigation, morphological and functional imaging studies, in order to establish the correct diagnosis and to assess the perioperative risks. The association of adrenocortical and adrenomedullary tumors with renal angiomyolipoma described in our case is not common. It can be sporadic or may result from inherited disorders or multiple neoplasia syndromes.