Endocrine Abstracts

Introduction: Adrenal adenomas development has been partially explained by aberrant adrenal expression of some hormone receptors. Klinefelter’s syndrome is a common cause of hypergonadotropic hypogonadism, which is characterized by increased serum LH level. In this case, we report a patient with Klinefelter syndrome, who was later diagnosed with a non secreting adrenal adenoma.

Case presentation: We report the case of a 57 years old patient, diagnosed with Klinefelter syndrome since the age of 34. He was treated with testosterone replacement therapy for only four years than stopped consulting. The patient was meanwhile treated for hypertension, atrial flutter and heart failure. He also had a history of hypokalemia. He presented to the emergency department for palpitations with dyspnea. Physical examination showed an obesity with BMI at 37.5 kg/m^². Blood pressure was EKG showed supraventricular tachycardia. Pulmonary embolism was suspected and a CT angiography was conducted, and a right adrenal tumor mesuring 13 mm was discovered. His plasma cortisol level was suppressed to 17 nmol/l after the 1-mg dexamethasone suppression test. Plasmatic normetanephrine and metanephrine levels were normal (respectively 0.52 nmol/l and <0.10 nmol/l). His plasma aldosterone concentration and renin activity were 29.9 pg/ml and 3.9 mUI/l, respectively, with a normal aldosterone to renin ratio (17). He was diagnosed as having a non secreting adrenal tumor.

Conclusion: Since untreated Klinefelter syndrome is characterized by increased serum LH, a possible contribution to adrenal tumors development is suggested. It has been described that some patients with untreated Klinefelter’s syndrome, later develop primary aldosteronism.