SUN-156 Adrenal Cortical Carcinoma in a Male Patient Who Presented with Classic Signs and Symptoms of Cushing’s Syndrome

Abstract

Background: Adrenal Cortical Cancer (ACC) is a rare malignancy with an annual incidence of 1-2 per million population. ACC is generally considered a highly malignant tumor and account for 0.05-2% of all cancer.Clinical case: A 32-year old male, newly diagnosed diabetic and hypertensive for 1 month, with a BMI of 37.91 kg/m2, presented with intermittent, vague, non-radiating flank pain. Within a month, patient started to develop weight gain associated with Cushingoid fascies, buffalo hump, elevated blood sugars, hypertension, and eventually developed purple striae in the abdomen associated with easy bruisability. Ultrasound was done which showed bilateral nephrolithiasis. Further work-up with CT-stonogram was done which showed bilateral nephrolithiasis and a 55x37x60mm heterogeneous mass above the left kidney in the area of the left adrenal gland. Further imaging with whole abdominal CT scan was done which revealed stable size of the mass in the region of the left adrenal gland with heterogeneous attenuation and enhancement.Initial hormonal work-ups done showed abnormal 1mg dexamethasone suppression test (874mmol/L N: 172-497mmol/L), but with normal aldosterone (73.22, N: <90pg/ml), renin (8.60pg/mL, N: 5-40pg/mL) and 24-hour urine metanephrines (31 mcg/24 hours N: 115-695mcg/24 hours). Serum K and blood glucose was managed accordingly and patient was cleared to undergo surgery. Patient underwent left unilateral laparoscopic adrenalectomy which revealed a 7cm soft, friable left adrenal mass with minimal hematoma on the antero-inferior portion of the adrenal gland, with active bleeding. Histopathology of the left adrenal mass revealed high-grade adrenal neoplasm, consistent with adrenocortical carcinoma.Postoperatively, patient did not have complications of hypo nor hyperglycemia, hypotension and serum potassium was stable. Hydrocortisone was given postoperatively in tapering doses. Repeat cortisol were done 24 and 48 hours post op which showed normal results (4.02ug/dL and 5.37 ug/dL respectively, N: 5-25 ug/dL). Patient was referred to Oncology service for Chemotherapy and was eventually discharged stable with home medications for his Diabetes mellitus.On follow-up, there was noted improvement on the signs and symptoms of Cushing’s syndrome, with no hypokalemia and better blood glucose control. Conclusion: Majority of adrenal mass are benign but a high index of suspicion for malignancy should always be part of the workup. The acuteness of symptoms and size of mass are important determinants of malignancy. Prior to adrenalectomy, perioperative endocrine therapy is a must. Adrenocortical carcinoma should be managed by a multidisciplinary specialist team including an endocrinologist, oncologist, surgeon, radiologist, and a histopathologist.