Endocrine Abstracts

Introduction: Pheocromocytomas are catecholamine-secreting tumors that arise from cromaffin cells of the adrenal medulla, probably occurring in less than 0.2% of pacients with hypertension. The objective of this report is to describe a case of norepinephrine-only secreting pheocromocitoma.

Case description: A 43-year-old female pacient presented with hypertension, recent onset diabetes and right adrenal mass measuring 3.5×2.7 cm on a CT scan. Hormonal tests showed normal basal cortizol level, normal cortisol level after 1 mg Dexamethasone test, high levels of aldosterone and renin, elevated normetanephrine levels (12xN) with normal metanephrine levels, high levels of chromogranin A. The normal metanephrine levels with elevated normetanephrine may be a characteristic of VHL syndrome. Thyroid ultrasound, thyroid hormones levels, PTH, calcitonin were normal. Genetic testing for VHL syndrome was made, results being not available for now. The pacient was treated with alpha blockers for several days, the beta-blocking drug was added and she underwent total adrenalectomy. The histological+IHC report confirm benign pheocromocitoma. Postoperative, the pacient had complete resolution of symptoms with normal blood pressure and resolution of diabetes.

Conclusion: A pheocromocytoma is a rare catecholamine secreting tumor. About 30% of pheocromocytomas occur as part of hereditary syndromes. The characteristic of pheocromocytomas associated with VHL syndrome is normetanephrine-only secretion. Genetic testing is important because can set appropriate follow-up and surveillance.