A Case Report of Severe Cushing's Syndrome Successfully Treated with Left Adrenal Embolization of the Adrenal Adenoma with 6 Month Follow up

Presentation Number: MON 405
Date of Presentation: April 3rd, 2017

Shazia Ahmad*1, Raquel Kristin Ong2 and Hassan Shawa1
1Albany Medical College, Albany, NY, 2Albany medical college, Albany, NY

Abstract

Background: Cushing’s syndrome is a disease characterized by a state of hypercortisolism, resulting in significant morbidity and mortality. Early diagnosis and treatment is essential. We report a very challenging case of adrenal Cushing’s syndrome that posed multiple diagnostic and treatment challenges and subsequently successfully treated with super-selective adrenal embolization.

Case Report: 35 year old morbid obese woman with Cushing’s syndrome was admitted to our hospital in septic shock secondary to necrotic pancreatitis and peri- pancreatic abscess. Prior to admission, the initial outpatient workup of Cushing’s syndrome was triggered after patient had experienced multiple fractures, 100 pound weight gain, and recurrent infections over 5 years. That work up revealed a random cortisol of 26.1ng/dL (nl < 22.4) and an ACTH less than 1.1pg/dL. 24-hour urine free cortisol was 50.5mcg/d (nl< 24), and repeat was 25.8 mcg/d. 1 am salivary cortisol was 1.386 (nl< 0.112) – further confirming hypercortisolism. CAT (CT) scan showed 3.5 cm left adrenal tumor. Patient was scheduled for laparoscopic left adrenalectomy as outpatient. However, she presented to our hospital with septic shock and multiple organ failure in the meantime. Patient was evalauted by Endocrine surgery and was considered a poor surgical candidate; therefore, she was initiated on medical management with Ketoconazole. She was then transitioned to Metyrapone because of ketoconazole’s diminished response due to decreased absorption with concomitant proton pump inhibitor administration. She underwent exploratory laparotomy, but adrenal glands could not be successfully accessed due to extensive fibrosis and inflammation. Patient eventually underwent super-selective embolization of the left adrenal gland adenoma by interventional radiology. She tolerated the procedure well. Metyrapone was discontinued and she was initiated on replacement dose of hydrocortisone. She lost almost 80 LBs and many of the Cushingoid features improved significantly within 3 months. Follow up CT abdomen showed diminishing size of left adrenal adenoma. Patient remained on hydrocortisone replacement in the meantime. Unexpectedly, patient presented with altered mental status 6 months after the procedure and was found to have massive hemorrhagic stroke. She was placed on comfort measures and unfortunately expired.

Conclusion: Cushing’s syndrome can present with significant morbidity and mortality. Surgery remains the treatment of choice once the source is identified. We present a rare case of adrenal Cushing’s syndrome treated successfully with super-selective adrenal embolization with biochemical remission for six months after the procedure. Adrenal embolization could be considered in patients with cortisol-producing adenomas when surgery is not an option.

 

Nothing to Disclose: SA, RKO, HS