MON-518 - Non-ACTH Dependent Cushing'S Syndrome Caused by Adrenocortical Carcinoma

Introduction:
Adrenocortical carcinoma is a rare, yet aggressive cancer stemming from the adrenal cortex. It may present with signs and symptoms of hormone hypersecretion or mass effect. It is found to be associated with hereditary cancer syndromes including Li Fraumeni, Lynch, multiple endocrine neoplasia and familial adenomatous polyposis. Here, we present a case of rapidly progressive adrenocortical carcinoma with normal DHEA-S levels.
Clinical

Case:
50-year-old woman with a medical history of newly diagnosed hypertension presented with worsening left sided abdominal pain, fatigue, weight gain, muscle weakness, and facial puffiness over the last two months. Physical exam showed that she was obese, with moon facies and abdominal striae. Outpatient evaluation revealed normal aldosterone/plasma renin activity, plasma metanephrines, and TSH. Hemoglobin A1C was 5.7%, ACTH < 5 pg/ml (NR 6-50), and AM cortisol 27 mcg/dl (NR 4-19). DHEA-S level was 89 µg/dL (30–512), which is not elevated as expected in a DHEA-S–producing tumor and not suppressed as would be typical of a cortisol-producing tumor. 24-hour urine free cortisol was 554 and 508 mcg/dl (NR 3-42) on two occasions. CT adrenal demonstrated a 5.9x5.5x5.9 cm left adrenal mass (37.4 HU) with heterogenous enhancement on the parenchymal phase with central areas of non-enhancement showing necrosis. It showed a 20% percent washout and 7% absolute washout. She underwent an open left adrenalectomy with pathology showing low grade adrenocortical carcinoma, invading the adrenal capsule. She was subsequently discharged on a steroid taper.

Conclusion:
This case highlights the importance of considering adrenocortical cancer in a patient with rapidly progressive Cushing's syndrome. It reinforces ensuring multidisciplinary care with endocrinology, surgery, internal medicine, and oncology to optimize disease outcomes given the rarity and aggressiveness of this disease. It is vital that a comprehensive metabolic/hormonal profile be done as adrenocortical carcinoma can have different steroid precursor production and evaluating isolated values like DHEA-S may be falsely reassuring.

*Unless otherwise noted, all abstracts presented at ENDO must not be released to the press or the public until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.*