Endocrine Abstracts

Introduction: Adrenocortical carcinomas (ACC) are rare and frequently aggressive tumors that may be functional (hormone-secreting) – causing Cushing’s syndrome and/or virilization, or nonfunctional – presenting as an abdominal mass or as an incidental finding. We describe a rare case of stage IV adrenocortical carcinoma which first manifested as treatment resistant Cushing’s syndrome.

Case report: In september of 2021, a 24-year-old female came with a complaint of acne and weight gain. During the process of anamnesis collection other symptoms characteristic to hypercortisolism were identified – rounding and flushing of the face, widening of the neck, abdominal weight gain, limb weakening and thinning, increase in blood pressure and thirst. In initial blood test results biochemical parameters testosterone and plasma metanephrines were within normal limits. Serum cortisol was elevated (1049,9 nmol/l), also secondary hypothyroidism was found. Low-dose dexamethasone test was performed and no suppression was observed, cortisol after suppression test remained high – 797 nmol/l. We confirmed autonomous cortisol secretion after disturbed circadian cortisol rhythm, high levels of the 24-hour urinary free cortisol, suppressed adrenocorticotropic hormone were found. Following that, chest, abdominal, pelvis computed tomography (CT) scan was performed and right adrenal mass was identified (90×62×127 mm), with metastases in the liver and in nearby lymph nodes. Interdisciplinary team decided on a treatment plan consisting of radical surgery followed by a postoperative chemotherapy treatment with mitotane. 2021-10-15 radical surgery was performed and she started the rehabilitation process. After surgery she felt better, she lost weight, her blood pressure became normal. 2021-10-28 histological test results came and adrenocortical carcinoma with metastases in liver was confirmed. 2021-11-29 treatment with doxorubicin, cisplatin and etoposide has begun, but after the first 3 days of treatment – chemotherapy induced cardiotoxicity appeared. She was hospitalized 2021-12-02. 2021-12-06 Abdominal CT scan showed tumor recurrence and progression. Due to hypokalemia, hypercortisolism – etomidate therapy was given, starting from 40 mg a day, through 8 h intravenously. 2021-12-22 mitotane was given, starting from 2000 mg per day. One more cycle of chemotherapy was tried, but her condition rapidly deteriorated and on 2022-01-05 she died.

Conclusion: Our case showed that despite the rarity of the tumor and young patient’s age it is important to perform further clinical evaluation in any patient with unexplained symptoms characteristic to hypercortisolism.