Endocrine Abstracts

Cushing’s syndrome (CS) is associated with a considerable risk of complications including thromboembolic events (TE). They occur mostly within the first 2–3 months postoperatively. When present before surgery, CS has high rates of perioperative mortality and morbidity. The benefit of steroidogenesis inhibitors after TE is not fully known. Furthermore, little guidance is available regarding TE assessment/management in CS. We report a case of a 34-year-old male admitted in our emergency department for a presyncope episode during minimum physical activity. He described severe fatigue that had progressively worsened over 3 months. He experienced progressive weight gain of 10 kg and the appearance of extensive purple trunk striae in the last year. His past medical history included hypertension, obesity and obstructive sleep apnea. His blood pressure was 100/78 mmHg, his heart rate 150 bpm and oxygen saturation was 99%. Laboratory tests revealed: hemoglobin 15 g/dl, leukocytosis 13,200/mm3, AST 259 U/L (15–37), ALT 653 U/L (16–63), indirect hyperbilirubinemia 1.20 mg/dl, D-dimer 70.370 ng/ml (<500), NT-proBNP 125 pg/ml, troponin T 663 ng/l. CT scan pulmonary angiography found extensive pulmonary embolism involving the right and left pulmonary arteries with extension to lobar arteries, also revealed a 35 mm solid tumor on his left adrenal gland. Lower limb color doppler ultrasound demonstrated deep venous thrombosis in the right popliteal vein. Anticoagulant therapy was initiated. The patient was hospitalized for treatment and further investigation. Elevated urinary free-cortisol(UFC) levels were noted (936 μg/24 h;1389 μg/24 h;28–213), ACTH level was undetectable (<5 pg/ml), salivary cortisol showed loss of circadian rhythm (32.8 nmol/l- 0800 h; 32.5 nmol/l-0011 h), and low dose dexa 1 mg plasma cortisol result was 26.4 μg/dl and low DHEA-S of 76 μg/dl (80–560). These results were compatible with ACTH-independent CS. He started on metyrapone to control the hypercortisolaemia (maximum 1500 mg 8–8 h). Ketoconazol was not an option given the elevated liver enzymes. There was an initial reduction in 24 hours UFC within a few weeks of initiation of metyrapone. However, the UFC level failed to normalize, after 7 months of medical therapy alone(UFC 706 μg/24 h).An uneventful laparoscopic left adrenalectomy was subsequently performed, with hydrocortisone started for empiric treatment of secondary adrenal insufficiency. Postoperative histopathology confirmed an adrenal adenoma. He completed 3 months of rivaroxabane. Presently, 5 months after surgery, he remains on hydrocortisone. Studies on medical therapy directed to lower cortisol values and their effects in TE risk are sparse. Additional studies are needed to reinforce well defined guidelines regarding CS and its thrombotic complications.